Cautionary Note On Forward-Looking Statements: This presentation contains forward-looking statements. All statements other than statements of historical facts contained in this presentation, such as statements regarding

Ascendis Pharma A/S January 2018

Cautionary Note On Forward-Looking

Statements: This presentation contains forward-looking statements. All statements other than statements of historical facts contained in this presentation, such as statements regarding our future results of operations and financial position,

including our business strategy, prospective products, availability of funding, clinical trial results, product approvals and regulatory pathways, collaborations, timing and likelihood of success, plans and objectives of management for future

operations, and future results of current and anticipated products, are forward-looking statements. These forward-looking statements are based on our current expectations and beliefs, as well as assumptions concerning future events. These statements

involve known and unknown risks, uncertainties and other factors that could cause our actual results to differ materially from the results discussed in the forward-looking statements. These risks, uncertainties and other factors are more fully

described in our reports filed with or submitted to the Securities and Exchange Commission, including, without limitation, our most recent Annual Report on Form 20-F filed with the SEC on March 22, 2017, particularly in the sections titled

"Risk Factors" and "Management's Discussion and Analysis of Financial Condition and Results of Operations". In light of the significant uncertainties in our forward-looking statements, you should not place undue

reliance on these statements or regard these statements as a representation or warranty by us or any other person that we will achieve our objectives and plans in any specified timeframe, or at all. Any forward-looking statement made by us in this

presentation speaks only as of the date of this presentation and represents our estimates and assumptions only as of the date of this presentation. Except as required by law, we assume no obligation to update these statements publicly, whether as a

result of new information, future events or otherwise after the date of this presentation. This presentation concerns product candidates that are or have been under clinical investigation and which have not yet been approved for marketing by the

U.S. Food and Drug Administration, European Medicines Agency or other foreign regulatory authorities. These product candidates are currently limited by U.S. Federal law to investigational use, and no representations are made as to their safety or

effectiveness for the purposes for which they are being investigated. Ascendis is a trademark that we use in this presentation. Any other trademarks appearing in this presentation are the property of their respective holders.

Company Overview Create best-in-class

rare disease products addressing unmet medical needs Apply TransCon technology to parent drugs with clinical proof-of-concept Expect higher development success rate compared to traditional drug development Endocrinology rare disease internal

pipeline and expected key 2018 milestones TransCon Growth Hormone for pediatric GHD: fliGHt (switch) Trial fully enrolled Q3 TransCon PTH for hypoparathyroidism: Phase 1 full data in Q2 TransCon CNP for achondroplasia: Phase 1 top-line data in Q4

Build leading positions for each of our endocrinology rare disease products with commercial focus on the U.S. and selected European markets Established high-value collaborations with Roche/Genentech in ophthalmology and Sanofi in diabetes As of

September 30, 2017, pro forma cash and cash equivalents of 222.5 million1 1 Reflects net proceeds from a follow-on offering announced on September 27, 2017, including underwriters' exercise of their overallotment option

TransCon Technology Overview Parent

drug is protected from receptor activation and clearance by transiently binding to the linker-carrier Parent drug distribution as original compound; Linker-carrier elimination mainly by renal clearance Following injection, the TransCon linker slowly

autohydrolyzes to release unmodified parent drug TransCon carrier TransCon linker Inactive parent drug Unmodified parent drug Receptor Renal clearance Linker cleavage only dependent on pH and temperature

Ascendis Approach to Product Innovation

1 Totals from FDA and EMA Orphan List, public & proprietary databases, advisors and conferences Unmet Medical Need Clinically Validated Parent Drug TransCon Technology Suitability Clearly Differentiated Product Established Clinical &

Regulatory Pathway Large Addressable Market Higher Value, Lower Risk Pipeline ~4,800 Orphan Drug Designations >500 Endocrine/Metabolic1

PRODUCT CANDIDATE PRIMARY INDICATION

DEVELOPMENT STAGE POTENTIAL WW MARKET1 WW COMMERCIAL RIGHTS TransCon Anti-VEGF Ophthalmology Not disclosed >$7 billion TransCon Peptides Diabetes Not disclosed >$1 billion PRODUCT CANDIDATE PRE IND PHASE 1 PHASE 2 PHASE 3 POTENTIAL WW MARKET1

WW COMMERCIAL RIGHTS TransCon hGH > $3 billion2 TransCon PTH > $2 billion3 TransCon CNP > $1 billion Building a Leading Company in Rare Diseases Internal Endocrinology Pipeline Hypoparathyroidism Pediatric Growth Hormone Deficiency

Achondroplasia 1 Based on market data and company estimates 2 Includes all indications 3 Based on treatment of ~25% of the U.S. patient population of ~80,000 patients Strategic Collaborations

TransCon Growth Hormone: Once-Weekly

Well established market primed for

disruption by a long-acting growth hormone product The Growth Hormone Market1 ~$3.5 billion in worldwide daily hGH sales and growing (2.4% CAGR) Fragmented market with same undifferentiated hGH molecule competing on differentiated formulations,

devices, services and access strategies Pediatric indications comprise ~90% of the market Indications for growth hormone treatment include: Growth Hormone Deficiency (GHD) ~50% of market Turner Syndrome Idiopathic short stature (ISS) Prader-Willi

Syndrome Small for Gestational Age (SGA) 1 Company research

Growth Hormone Deficiency: Clinical

Manifestations 1 BMC Endocrine Disorders 2012, 12:26 2 J Clin Endocrinol Metab 2006, 91: 1621-1634 Long-acting GH must fully mimic daily hGH to address the totality of the disease PEDIATRIC Growth Hormone Deficiency1 Growth failure Increased

and abnormal fat distribution (especially truncal fat mass) Abnormal metabolic profile Impaired exercise capacity ADULT Growth Hormone Deficiency2 Trunk fat accumulation and decrease in lean body mass Decreased bone mineral density Dyslipidemia

Increased cardiovascular mortality and morbidity Decreased quality of life

Increased adherence Improved growth

response Doses missed per week 3 2 p< 0.001 p< 0.01 4 3 2 1 0 1 Height Velocity SDS Poor adherence with daily growth hormone therapy is associated with reduced height velocity and impaired quality of life1 Daily Growth Hormone:

The Problem 1 PLoS ONE 2011, 6(1): e16223 2 Clinical Therapeutics 2008, 30(2): 307-316 Reduced frequency of administration is associated with better adherence2 In the first year, two of three patients miss >1 injection on average per

TransCon Growth Hormone: The

Opportunity Predictable release of parent drug in the body hGH hGH TransCon carrier TransCon linker Inactive Prodrug Creation of TransCon prodrug Once-weekly prodrug releasing unmodified hGH designed to mimic daily hGH: Tissue distribution

Physiological levels Therapeutic effects: efficacy, safety and tolerability

Growth Comparable to a Daily hGH in

Phase 21,2 Same weekly dose 11.5 14.5 0.14 mg 0.21 mg 0.30 mg 0.21 mg Dose (hGH/kg/week): 1 Intergroup differences not statistically significant 2 JCEM, DOI: 10.1210/jc.2016-3776 3 Conducted with a previous lower strength version of TransCon Growth

Hormone 26-week treatment period Two thorough PK/PD assessments at weeks 1 and 13 TransCon Growth Hormone3 Genotropin

Dose Proportional IGF-1 in Phase 21

Days (Week 13) IGF-1 SDS (+ SEM) 0 1 2 3 4 5 6 7 Transient point values of IGF-1 SDS > +2.0 have been observed in a small number of patients primarily at the highest dose level 1 JCEM, DOI: 10.1210/jc.2016-3776

Comparable hGH Levels in Phase 21

Maximum hGH concentration comparable between equivalent weekly doses of TransCon Growth Hormone and a daily hGH Days (Week 13) hGH serum concentration (+SEM, ng/ml) 1 JCEM, DOI: 10.1210/jc.2016-3776

Comparable Safety to a Daily hGH in

Phase 21 >1100 TransCon Growth Hormone injections administered in the phase 2 pediatric study No reports of lipoatrophy or nodule formation No Serious Adverse Events related to study drug Immunogenic profile comparable to a daily hGH Injection

site tolerability comparable to a daily hGH Adverse events consistent with a daily hGH therapy observed and not different between cohorts No occurrence of neutralizing antibodies Low incidence of low-titer non-neutralizing antibodies 1 JCEM, DOI:

10.1210/jc.2016-3776

TransCon Growth Hormone Phase 3

Update FDA and EMA support size and scope of program for pediatric GHD filing Database lock for filing package Q3 2019 Switch trial Enrollment completion ~Q3 2018 Extension trial (n= ~300) Regulatory Filings Pivotal trial Fully enrolled Data

expected Q1 2019 Ongoing

Phase 3 heiGHt Trial Ongoing Key

Inclusion Criteria Prepubertal children with GHD Height SDS -2.0 IGF-1 SDS -1.0 GHD with 2 GH stim. tests (GH 10 ng/mL) Bone age 6 months behind chronological TransCon Growth Hormone (0.24 mg/kg/week) Genotropin

(34 g/kg/d = 0.24 mg/kg/week) Key Endpoints Annualized height velocity (HV) at 52 weeks (primary endpoint) Annualized HV at earlier time points Change in HT SDS over 52 weeks Change in serum IGF-1/IGFBP-3 levels Change in IGF-1 SDS and

IGFBP 3 SDS Normalization of IGF-1 SDS Long-Term Extension Trial Week 1 Week 5 Week 13 Week 52 Week 26 Week 39 >160 Treatment-na ve children with GHD (2:1 randomization) VISIT SCHEDULE Screening 6 weeks

Auto-Injector Designed to Improve

Adherence Auto-injector introduction during extension study and for commercial launch Simple operation with few user steps A single low-volume (<0.60 mL) injection for patients less than 60kg Small needle, comparable to daily hGH (31G, 4mm) Room

temperature storage No waste due to empty-all design Bluetooth connectivity enabled for automatic data capture Device lifetime at least 4 years Key Features

Improve Adherence Through Feedback

& Intervention Secure Cloud-Based Central Database TransCon hGH Auto-Injector Automated Data Capture of Dose and Injection Time Integrating with a Connected Healthcare System

Efficacy Safety (including

immunogenicity) Tolerability TransCon Growth Hormone Target Product Profile Comparable to Daily Growth Hormone Weekly subcutaneous administration Small injection volume (31G needle) Room temperature storage Device Easy to use Automatic data capture

TransCon Growth Hormone: Highlights

Potential best-in and first-in class long-acting growth hormone in pediatric GHD Trial phase 3 top-line results expected Q1 2019 Trial expected to be fully enrolled Q3 2018 Auto-injector developed and on-track for introduction in Trial Improving

adherence through integrated automatic data capture and connected healthcare system Commercial-scale manufacturing and supply chain established Multiple patent filings provide potential protection into 2038

TransCon PTH: Once-Daily for

Hypoparathyroidism: Serious Unmet

Medical Need1 Parathyroid hormone (PTH) regulates calcium/phosphate homeostasis Calcium is essential for muscle, skeletal, neurological and cardiac function Hypoparathyroidism (HP) is a rare disease characterized by deficient or absent PTH Results

in low calcium and increased phosphate blood levels Most common cause (~75%) is inadvertent removal or damage to parathyroid glands during thyroid surgery Approximately 80,000 patients in the U.S. HP results in diverse range of physical, cognitive,

emotional symptoms and reflects a high burden across the healthcare system Symptoms include muscle cramps, tetany, seizure, cardiac abnormalities, bronchospasm, laryngospasm and altered mental status 1 Nature Reviews 2017, vol 3, 1-20

Hypoparathyroidism: Burden of

Illness Calcium multiple times daily and vitamin D is current standard of treatment Despite current treatment, study showed1: 72% of patients experienced >10 symptoms* in the preceding 12 months Symptoms were experienced for a mean of 13 9

hours/day Hospital stays or emergency department visits were required by 79% of patients 85% reported an inability to perform household activities 20% experienced a disease-associated change in employment status Patients on standard treatment

compared to healthy controls have2: 4-fold increased risk of hospitalization due to seizure 4-fold increased risk of renal diseases (calcifications and renal insufficiency) * Fatigue, muscle pain or cramping, paresthesia, tetany, joint or bone pain,

pain/heaviness/weakness in extremities, brain fog/mental lethargy, inability to focus or concentrate, memory loss or forgetfulness, sleep disturbances, anxiety and depression 1 Endocrine Practice; 2014, Vol. 20 pp. 671-679; results of Paradox survey

of patients (n=374) 2 J Bone Miner Res 2013, 28, 2277-2285, results of 688 patient survey on standard treatment compared to age- and gender-matched control

HP Treatment Strategies Are

Evolving1,2 Conventional treatment by calcium and vitamin D does not fully replace the functions of PTH and can lead to: Short-term complications of hypocalcemia, hypercalcemia and hypercalciuria Long-term complications of impaired renal function

and extraskeletal calcifications Once-daily Natpara/Natpar has been approved in the U.S. and Europe as an adjunct to calcium and vitamin D to control hypocalcemia in HP patients Does not fully address all aspects of the disease, including

hypocalcemia, hypercalcemia, hypercalciuria and bone turnover TransCon PTH designed to address all aspects of the disease by normalizing: blood/urinary calcium levels, serum phosphate and bone turnover 1 Front. Endocrinol., 2017, 7:172 2 Nature

Reviews, 2017, vol 3, 1-20

FDA Perspective on Optimal PTH PK

Profile1 1 FDA presentation Natpara Advisory Committee September 12, 2014 Natpara QD provides dose-dependent increase in serum calcium for ~24 hours Natpara QD effect on urinary calcium excretion is short-lived (10-12 hours) as kidney reabsorption

of calcium follows PK profile

Continuous PTH Infusion Led to

Improved Outcomes Desired Treatment Outcomes in HP Natpara Once-daily1, 2 PTH (1-34) Infusion3-9 Increase serum calcium Reduce pill burden Normalize urinary calcium excretion X Reduce hypercalcemia X Reduce hypocalcemia X Normalize serum phosphate

(high-normal range) Normalize bone turnover X (cortical bone loss) NIH clinical trials demonstrated superiority of continuous infusion > twice daily injections > once daily injections 1 Natpara Product Label 2 J Clin Endocrinol Metab 2016,

101(7): 2742-2750 3 JAMA 1996, 276(8): 631-636 4 J Clin Endocrinol Metab 1998, 83(10): 3480-3486 5 J Clin Endocrinol Metab 2003, 88(9): 4214-4220 6 J Clin Endocrinol Metab 2008, 93(9): 3389-3395 7 J Clin Endocrinol Metab 2011, 96(11): 3308-3312 8 J

Clin Endocrinol Metab 2012, 97(2): 391-399 9 J Pediatr 2014, 165(3):556-563

TransCon PTH Design Predictable

release of parent drug in the body PTH(1-34) PTH(1-34) TransCon carrier TransCon linker Inactive Prodrug Creation of TransCon prodrug TransCon PTH is a sustained-release prodrug, designed to provide stable free PTH levels in the physiological range

for 24 hours a day TransCon PTH designed to normalize: blood/urinary calcium levels, serum phosphate and bone turnover

TransCon PTH Phase 1 Trial Design

Randomized, placebo controlled, single and multiple ascending dose trial to evaluate the safety, tolerability, PD and PK of TransCon PTH in healthy adults Primary Objective: To assess the safety and tolerability of single and 10 multiple daily doses