NEW SHARE-BASED INCENTIVE PROGRAM

Copenhagen, Denmark, July 29, 2019 – Orphazyme A/S (ticker: ORPHA.CO), a biopharmaceutical company dedicated to developing treatments for patients living with rare diseases, today announces that the Company introduces a new share-based incentive program (the “LTIP”). The LTIP has been implemented in accordance with Orphazyme’s Remuneration Policy, including the guidelines for incentive pay adopted at Orphazyme’s annual general meeting on March 27, 2019.

Under the LTIP, the Executive Management as well as certain key employees of Orphazyme have subscribed for or acquired a number of shares (“Investment Shares”). The Board of Directors may decide to offer other current or new employees of Orphazyme to participate in the LTIP.

The participants may be allocated a number of shares in Orphazyme (“Performance Shares”) at a price per Performance Share of DKK 1 at the end of a vesting period of up to four (4) years from the date of grant (the “Performance Shares Holding Period”). The number of Performance Shares shall be proportional to a potential increase in the price of Orphazyme’s shares at the time of vesting compared to the price at the date of the grant. The potential increase in the price of Orphazyme’s shares will be calculated as the volume weighted average share price as quoted on Nasdaq Copenhagen during the 10 trading days preceding the date of expiry of the Performance Shares Holding Period and the date of the grant, respectively. Performance Shares will be allocated on a linear scale with maximum allocation triggered by an 80% increase in share price, whereas no Performance Shares will be allocated, if the price of Orphazyme’s shares has increased 20% or less at the date of expiry of the Performance Shares Holding Period. Among other things, vesting is also subject to the participants having maintained ownership of their Investment Shares and continued employment at the time of vesting. Based on the number of Investment Shares held by the participants, a total maximum of up to 125,000 Performance Shares may be granted at the time of vesting.

Further, the participants may also be allocated a number of shares in Orphazyme (“Matching Shares”) at a price per Matching Share of DKK 1 after a period of up to one (1) year from the grant of the Matching Shares. The number of Matching Shares shall be equal to the number of Investment Shares held by the participant and vesting will be subject to the participants having maintained ownership of their Investment Shares and continued employment at the time of vesting. Based on the number of Investment Shares held by the participants, a total maximum of up to 31,250 Matching Shares may be granted at the time of vesting.

The theoretical market value of the LTIP has been estimated at approximately DKK 5.5 million based on a Monte Carlo model with a four-year volatility estimate of 53% and an expected dividend yield of 0%. This implies an expected number of Performance Shares to be allocated of 22,638.