Notice to convene Annual General Meeting

Orphazyme A/SCompany announcementNo. 31/2022www.orphazyme.comCompany Registration No. 32266355

Copenhagen, Denmark, June 7, 2022 – Orphazyme A/S (ORPHA.CO) (the “Company”), today announces the notice to convene the Company’s Annual General Meeting to be held on:

Wednesday, June 29, 2022, at 2:00 PM (CEST)

at the offices of Gorrissen Federspiel, Axeltorv 2, DK-1609 Copenhagen V.

The notice to convene the Annual General Meeting, including Appendix 1: Candidates for the Board of Directors is attached.

Additional information about the Annual General Meeting is available on the Company’s website: https://orphazyme.gcs-web.com/annual-general-meeting-0

For additional information, please contact

Orphazyme A/S

Anders Vadsholt, Chief Executive Officer and Chief Financial Officer: +45 28 98 90 55

Forward-looking statement This company announcement may contain certain forward-looking statements under the U.S. Private Securities Litigation Reform Act of 1995 and otherwise, including forward-looking statements about the Company’s annual meeting, annual and interim reports and timing thereof. Although the Company believes its expectations are based on reasonable assumptions, all statements other than statements of historical fact included in this company announcement about future events are subject to (i) change without notice and (ii) factors beyond the Company’s control, including pursuant to regulatory or judicial intervention. Except as required by law, the Company assumes no obligation to update these forward-looking statements publicly, or to update the reasons actual results could differ materially from those anticipated in the forward-looking statements, even if new information becomes available in the future.

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