Agios Reports Fourth Quarter and Full Year 2019 Financial Results Fourth Quarter TIBSOVO Net Revenue of $19.6M and $59.9M for Full Year 2019; Full Year 2020 U.S. TIBSOVO Net Revenue Expected to be Between $105-115 Millio

Agios Reports Fourth Quarter and Full Year 2019 Financial Results

Fourth Quarter TIBSOVO Net Revenue of $19.6M and $59.9M for Full Year 2019;

2020 U.S. TIBSOVO Net Revenue Expected to be Between $105-115 Million

New Clinical Data from Mitapivat in Thalassemia and Sickle Cell

Disease to be Submitted for

Presentation at the European Hematology Association Annual Congress in June

Investigational New Drug Submission for AG-946, a Next Generation PKR Activator, Expected in

CAMBRIDGE, Mass., February 13, 2020 Agios Pharmaceuticals, Inc. (NASDAQ: AGIO) today reported business

highlights and financial results for the fourth quarter and year ended December 31, 2019. In addition, Agios highlighted key 2020 corporate milestones and data presentations for its clinical development programs.

On the heels of a busy and productive 2019, I m more confident than ever in the strength of our team and our ability to make a meaningful impact on

the lives of patients through great science, a deep pipeline and differentiated therapies, said Jackie Fouse, Ph.D., chief executive officer at Agios. In 2020, our clinical development team is focused on advancing our Phase 3 PK

deficiency studies in order to submit a new drug application in 2021, finalizing our pivotal development plan for the PK activation program in thalassemia and establishing

proof-of-concept in sickle cell disease. In addition, we are driving enrollment in several Phase 3 studies for our IDH inhibitors in both malignant hematology and solid

tumors. Our commercial team is focused on achieving an ambitious revenue target for TIBSOVO and increasing market development activities in preparation for a potential launch in PK

ANTICIPATED 2020 KEY MILESTONES

Agios expects the following key milestones in 2020:

Hematologic Malignancies

Rare Genetic Diseases

FOURTH QUARTER AND FULL YEAR 2019 FINANCIAL RESULTS

Revenue: Total revenue for the fourth quarter of 2019 was $35.4 million, which includes $12.9 million in collaboration revenue,

$19.6 million of net product revenue from sales of TIBSOVO and $3.0 million in royalty revenue from net global sales of

IDHIFA under our collaboration agreement with Celgene. This compares to $30.0 million for the fourth quarter of 2018, which included $18.4 million in collaboration

revenue, $9.4 million of net product revenue from U.S. sales of TIBSOVO and $2.2 million in royalty revenue from net global sales of IDHIFA . Total revenue for the year ended December 31, 2019 was $117.9 million compared to $94.4 million for the year ended December 31, 2018. The increase in 2019 revenue was

primarily driven by net U.S. sales of TIBSOVO and were offset by a decline in collaboration revenue due to the recognition of a milestone from Celgene and the upfront payment from CStone in

Cost of Sales: Cost of sales were $0.3 million for the fourth quarter of 2019 compared to $0.7 million for the fourth quarter of

2018, and $1.3 million for the year ended December 31, 2019 compared to $1.4 million for the comparable period in 2018.

Development (R&D) Expenses: R&D expenses were $106.2 million for the fourth quarter of 2019 compared to $93.8 million for the fourth quarter of 2018 and $410.9 million for the year ended December 31, 2019

compared to $341.3 million for the comparable period in 2018. The increase in R&D expense was primarily attributable to clinical trial activity for mitapivat in PK deficiency and thalassemia;

start-up costs for the vorasidenib Phase 3 INDIGO study in low-grade glioma, including required clinical pharmacology studies and companion diagnostic development; and

ongoing enrollment in the TIBSOVO Phase 3 AGILE and HOVON frontline AML combination studies. R&D expense also increased as a result of ongoing research efforts across our discovery

Selling, General and Administrative (SG&A) Expenses: SG&A expenses were $34.8 million for the fourth quarter of

2019 compared to $31.9 million for the fourth quarter of 2018, and $132.0 million for the year ended December 31, 2019 compared to $114.1 million for the year ended December 31, 2018. The increase in SG&A

expense was primarily attributable to increased investment in marketing activities in preparation for the potential launch of mitapivat and personnel costs related to increased headcount to support growing operations.

Net Loss: Net loss was $102.4 million for the fourth quarter of 2019 compared to

$91.8 million for the fourth quarter of 2018, and $411.5 million for the year ended December 31, 2019 compared to a net loss of $346.0 million for the year ended December 31, 2018.

Cash Position and Guidance: Cash, cash equivalents and marketable securities as of December 31, 2019 were $717.8 million compared

to $805.4 million as of December 31, 2018. The change in cash was primarily driven by expenditures to fund operations of $464.4 million offset by the net proceeds of $277.2 million from the November follow-on offering and cash inflows of $99.3 million from product sales, stock option exercises, royalty revenue, and collaboration reimbursements and milestones. The company expects that its cash, cash

equivalents and marketable securities as of December 31, 2019, together with anticipated product and royalty revenue, anticipated interest income, and anticipated expense reimbursements under our collaboration and license agreements, but

excluding any additional collaboration-related payments, will enable the company to fund its anticipated operating expenses and capital expenditure requirements through at least the end of 2021.

CONFERENCE CALL INFORMATION

Agios will host a conference

call and live webcast with slides today at 8:00 a.m. ET to discuss fourth quarter and full year 2019 financial results and recent business activities. To participate in the conference call, please dial 1-877-377-7098 (domestic) or 1-631-291-4547

(international) and referring to conference ID 4195413. The live webcast can be accessed under Events & Presentations in the Investors section of the company s website at www.agios.com. The archived webcast will be

available on the company s website beginning approximately two hours after the event.

About Agios/Celgene Collaboration

IDHIFA (enasidenib) and AG-270 are part of our collaboration

with Celgene Corporation, a wholly owned subsidiary of Bristol-Myers Squibb Company. Under the terms of our 2010 collaboration agreement focused on cancer metabolism, Celgene has worldwide development and commercialization rights for IDHIFA . Agios continues to conduct certain clinical development activities within the IDHIFA development program and is eligible to receive

reimbursement for those development activities and up to $80 million in remaining milestone payments, and royalties on any net sales. Celgene and Agios are currently co-commercializing

IDHIFA in the U.S. Celgene will reimburse Agios for costs incurred for its co-commercialization efforts. AG-270

is part of a 2016 global research collaboration agreement with Celgene focused on metabolic immuno-oncology. Celgene has the option to participate in a worldwide 50/50 cost and profit share with Agios, under which Agios is eligible for up to

$169 million in clinical and regulatory milestone payments for the program.

Agios is focused on discovering and developing novel investigational medicines to treat malignant hematology, solid tumors and rare genetic diseases through

scientific leadership in the field of cellular metabolism. In addition to an active research and discovery pipeline across these three therapeutic areas, Agios has two approved oncology precision medicines and multiple

first-in-class investigational therapies in clinical and/or preclinical development. For more information, please visit the company s website at www.agios.com.

Cautionary Note Regarding Forward-Looking Statements

This press release contains forward-looking statements within the meaning of The Private Securities Litigation Reform Act of 1995. Such forward-looking

statements include those regarding Agios plans, strategies and expectations for its and its collaborator s preclinical, clinical and commercial advancement of its drug development programs including TIBSOVO (ivosidenib), IDHIFA (enasidenib), mitapivat, vorasidenib, AG-270,

AG-636 and AG-946; the potential benefits of Agios product candidates; its key milestones and guidance for 2020; its plans regarding future data presentations; its

financial guidance regarding the period in which it will have capital available to fund its operations; and the potential benefit of its strategic plans and focus. The words anticipate, expect, goal,

hope, milestone, plan, potential, possible, strategy, will, vision, and similar expressions are intended to identify forward-looking statements, although

not all forward-looking statements contain these identifying words. Such statements are subject to numerous important factors, risks and uncertainties that may cause actual events or results to differ materially from Agios current expectations

and beliefs. For example, there can be no guarantee that any product candidate Agios or its collaborators is developing will successfully commence or complete necessary preclinical and clinical development phases, or that development of any of

Agios product candidates will successfully continue. There can be no guarantee that any positive developments in Agios business will result in stock price appreciation. Management s expectations and, therefore, any forward-looking

statements in this press release could also be affected by risks and uncertainties relating to a number of other important factors, including: Agios results of clinical trials and preclinical studies, including subsequent analysis of existing

data and new data received from ongoing and future studies; the content and timing of decisions made by the U.S. FDA, the EMA or other regulatory authorities, investigational review boards at clinical trial sites and publication review bodies;

Agios ability to obtain and maintain requisite regulatory approvals and to enroll patients in its planned clinical trials; unplanned cash requirements and expenditures; competitive factors; Agios ability to obtain, maintain and enforce

patent and other intellectual property protection for any product candidates it is developing; Agios ability to maintain key collaborations; and general economic and market conditions. These and other risks are described in greater detail

under the caption Risk Factors included in Agios public filings with the Securities and Exchange Commission. Any forward-looking statements contained in this press release speak only as of the date hereof, and Agios expressly

disclaims any obligation to update any forward-looking statements, whether as a result of new information, future events or otherwise, except as required by law.

Consolidated Balance Sheet Data

Consolidated Statements of Operations Data

( in thousands, except share and per share data)

Holly Manning, 617-844-6630

Associate Director, Investor Relations

Jessica Rennekamp, 857-209-3286

Associate Director, Corporate Communications