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 R&D Update

September 30, 2016 Exhibit 99.1

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, 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. These presentation materials include presentations from Drs. Barbara

Lippe, Dolores Shoback and Cathleen Raggio, who are experts in the fields in which they are presenting. They are providing background information about certain diseases, but their views do not necessarily represent those of the Company.

Vision 20/20 Jan Mikkelsen, President

Today's Agenda 8:30-8:45

a.m.Vision 20/20 & TransCon Technology (Jan Mikkelsen) 8:45-9:00 a.m.Pediatric Growth Hormone Deficiency (Barbara Lippe, M.D.) 9:00-9:20 a.m. TransCon Growth Hormone Program (Jonathan Leff, M.D.) 9:20-9:40 a.m. Q&A 9:40-9:45 a.m. TransCon

Treprostinil (Kennett Sprog e, Ph.D.) 9:45-10:00 a.m. Hypoparathyroidism (Dolores Shoback, M.D.) 10:00-10:20 a.m.TransCon PTH Program (David B. Karpf, M.D.) 10:20-10:35 a.m. Achondroplasia (Cathleen Raggio, M.D.) 10:35-10:55 a.m.TransCon CNP

Program (Jonathan Leff, M.D.) 10.55-11:25 a.m. Q&A 11:25-11:30 a.m. Closing Remarks

Vision 20/20: Building a Leading Rare

Disease Company Create best-in-class rare disease products addressing unmet medical needs by applying TransCon technology to parent drugs with clinical proof-of-concept Improve efficacy, safety and/or convenience Expected higher development success

rate compared to traditional drug development Endocrinology rare disease franchise Advance the company's pipeline of three rare disease endocrinology product candidates Obtain regulatory approval for and launch at least two products with $1+

billion market potential between 2020 and 2024 Next rare disease therapeutic area Identify at least three high value product opportunities in a second therapeutic area Initiate clinical development of the first product opportunity by 2020 Build an

integrated commercial business focused on the U.S. market based on internally developed best-in-class rare disease products

TransCon Technology Predictable release

of parent drug in the body Parent Drug Parent Drug TransCon carrier TransCon linker Inactive Prodrug Predictable release of unmodified parent drugs Parent drugs can be proteins, peptides or small molecules TransCon carrier enables both systemic and

localized drug exposure Maintains same mode-of-action of parent drug molecule Release of parent drug supporting up to half-yearly administration TransCon products eligible for new composition of matter IP claims Creation of TransCon

TransCon Linker Self-cleaving linkers

that autohydrolyze at a predictable rate Linker release only dependent on pH and temperature (in vitro / in vivo correlation) Linkers are stable under storage conditions (low pH and temperature or lyophilized) Sustained release of drug initiated

upon injection TransCon linker is eliminated with the carrier No adverse effects in repeat dose toxicology studies Trigger group Temporary linkage Permanent linkage Carrier Parent drug

Patent Filing Strategy Protection of

TransCon Technology Layered Protection of Product Concepts Broad patent portfolio covering reversible linkers, carriers and processes in major markets Linker claims directed at both chemical and functional aspects Linker families cover most drug

classes and help to establish strongest position to protect pipeline and value Multiple patent concepts protect each product opportunity Example of TransCon Growth Hormone covered by 12 different patent families including: Composition of matter,

product concept, dosing regimen, device, linker and branched carrier conjugates Patent protection expected until 2036

Multi-level Patent Portfolio Protection

69 Issued Patents > 200 Patent Applications Pending Product Concepts TransCon Linkers Dose Regimen Process Composition of Matter Device TransCon Carriers

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 4,758 Orphan Drug Designations 544 Endocrine/Metabolic1

Expertise in endocrinology

(clinical, regulatory, medical affairs) Concentrated prescriber audience Small, focused sales force Patient-centric support system Reimbursement and payor knowledge Distribution networks TransCon CNP TransCon Growth Hormone Expected Synergies of

Therapeutic Focus TransCon PTH

Product Candidate Primary Indication

Development Stage Potential WW Market1 WW Commercial Rights TransCon Growth Hormone Growth hormone deficiency Phase 3 > $3 billion TransCon PTH Hypoparathyroidism Pre-IND > $2 billion2 TransCon CNP Achondroplasia Pre-IND > $1 billion

TransCon Ranibizumab Ophthalmology Not disclosed > $7 billion TransCon Peptides Diabetes Not disclosed > $1 billion TransCon Treprostinil PAH Phase 1 > $1 billion Partnering Opportunity Endocrinology Ophthalmology CV Internal Rare Disease

Endocrinology Pipeline Current/Potential Strategic Collaborations 2 Based on treatment of ~25% of the U.S. patient population of ~77,000 patients. Building a Leading Company in Rare Diseases 1 Based on market data and company estimates

Today's Agenda 8:30-8:45

a.m.Vision 20/20 & TransCon Technology (Jan Mikkelsen) 8:45-9:00 a.m.Pediatric Growth Hormone Deficiency (Barbara Lippe, M.D.) 9:00-9:20 a.m. TransCon Growth Hormone Program (Jonathan Leff, M.D.) 9:20-9:40 a.m. Q&A 9:40-9:45 a.m. TransCon

Treprostinil (Kennett Sprog e, Ph.D.) 9:45-10:00 a.m. Hypoparathyroidism (Dolores Shoback, M.D.) 10:00-10:20 a.m.TransCon PTH Program (David B. Karpf, M.D.) 10:20-10:35 a.m. Achondroplasia (Cathleen Raggio, M.D.) 10:35-10:55 a.m.TransCon CNP

Program (Jonathan Leff, M.D.) 10.55-11:25 a.m. Q&A 11:25-11:30 a.m. Closing Remarks

Recombinant Human Growth Hormone

Life cycle progress toward the future Barbara Lippe, MD Professor Emerita of Pediatrics David Geffen School of Medicine at UCLA

What is GH deficiency and why do we

treat it? ...7 years later following treatment Tom Thumb 16 Months

Absence of any pituitary GH

secretion from infancy results in a normally proportioned "little person" ~ 70% of his/her expected adult height ~ 1/4000 children are born with or acquire GH deficiency Continuous treatment from early childhood can restore height to

near or at genetic potential and correct metabolic abnormalities Delayed/late therapy jeopardizes attaining full height potential Ultimate outcome depends on adherence, persistence, and responsiveness Background on GHD

Adapted from: Roelfsema V, et al. J

Am Soc Nephrol. 2001. IGF-1: insulin-like growth factor-1 SRIF: somatostatin, GHRH; growth hormone-releasing hormone Where does GH come from? How does it work?

Most common cause is

"idiopathic" = we're idiots because it's really pathologic Absence of a gene or genes in the pathway to GH synthesis or secretion Combination of congenital malformations of the brain Pituitary tumor (i.e. craniopharyngioma)

Radiation to the head as therapy for other conditions Head trauma Infections Other rare causes What causes failure to secrete enough GH?

Key Milestones Milestones in GH

treatment 1947 UCSF scientists isolate GH from pituitary Injections in dwarf children were "successful" Some physicians provided pituitary extracted GH GH extraction centralized at the NHPP; sent to hundreds of US endocrinologists ~

7,700 children were treated Creutzfeldt-Jakob Disease diagnosed in 3 of ~ 7,700 GH treated patients, years after GH therapy Extracted GH no longer manufactured FDA approves Protropin with IM injections, 3x/week 3Q 15 Genentech's

"Protropin", recombinant methionyl growth hormone was under FDA review 1963 - 1985 Spring 1985 Summer 1985 1985

Key Milestones Milestones in GH

treatment (cont'd) 1985 -1998 Progression to SC administration Efficacy with daily dosing Multiple companies enter market Improved body comp in GH deficient adults Liquid formulations, small needles and devices New indications for pediatric

and adult patients (e.g., Turner, short stature, Noonan, HIV body comp) First LA GH - Nutropin Depot Difficult to reconstitute, single use vials, large 21G needles Frequency of administration not ideal (bimonthly/monthly) Efficacy not as good as

daily (~ 7.8 cm/year) Withdrawn in 2004 Shift in payer and formulary control Gradual closure of registries More physicians in private practice Families are more "on their own" Outcomes still not maximized and expectations often not met

Adherence a major barrier partly due to frequency of injections Spring 1999 2000 - present

Why do we still need innovation? To

optimize adherence and compliance so that maximum growth can be achieved with the least amount of "mental" stress and "physical inconvenience"

Rosenfeld and Bakker. Endocr Pract.

2008; 14, 143-154 What are causes of patient non-adherence? Compliance means conforming Compliance Survey for GH How often . ? Forgot to take it Forgot to renew prescription Forgot to order the next refill Experience side effects Not able to

pay for it Away from home or traveling Illness or not feeling well enough Special event Needed a "vacation" or break from taking medication Noncompliance = answer "never" to 7 questions 64% of parents, 77% of teens,

and 65% of adults with GHD are nonadherent with treatment If switching from daily to weekly can motivate patients and families to be involved in what is a "less arduous" treatment they may become more compliant and turn into adherent

patients/families who are more motivated and involved with treatment and more comfortable with injections. Adherence is "steady devotion"

"Drugs don't work in

patients who don't take them" C. Everett Koop, MD Former Surgeon General

Depot formulations (e.g., Nutropin

Depot, LB03002) Covalent pegylation (e.g., Pfizer, Ambrx, Novo, GenSci's "Jintrolong") Hybrid molecules/fusion proteins (e.g., TEVA TV-1106, Versartis VRS-317, Novo somapacitan, LAPSrhGH/HM10560A [Hamni Pharma], GX-H9 [Handok,

Genexine, Inc.) Prodrug/sustained release (Ascendis TransCon Growth Hormone) Previous and current approaches to long-acting GH formulation

Provides all the benefits of

naturally secreted hGH Free GH molecule reaches all the target tissues Small volume administered with a very small needle Easy to administer with a user friendly device Room temperature stability Pain "free" (comparable

safety/tolerability to daily GH) Lack of immunogenicity (comparable to daily GH) What are the characteristics of an effective long-acting GH?

Frequency Pros Cons Twice Monthly

Fewer injections Confusing week on/off schedule Larger injection volumes, which may result in 2 shots/dose Less clinical experience Weekly Easy regimen to schedule Less frequent injections Less clinical experience than daily Daily Long track record

of safety/efficacy Patients don't like daily shots What is the preferred dosing regimen? Earlier treatment ensures better treatment outcomes

And this is how the child measures

it The goal Everyone should be able to reach the bar

TransCon Growth Hormone: Once-Weekly

Replacement Therapy Jonathan Leff, M.D. Chief Medical Officer

Higher compliance 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 compliance with daily growth hormone therapy is associated with reduced height velocity and impaired quality of life1 Poor Compliance

Reduces Treatment Outcomes 1 PLoS ONE 2011, 6(1): e16223 2 Clinical Therapeutics 2008, 30(2): 307-316 Reduced frequency of administration is associated with better compliance2 In the first year, two of three patients miss >1 injection on average

Desired Target Product Profile

Efficacy Safety (including immunogenicity) Tolerability Comparable to Daily Growth Hormone = Weekly subcutaneous administration Single injection/dose Convenience 31G needle Room temperature storage Device Easy to use Empty-all design (controlled

Mode of Action of TransCon Growth