Key Clinical Message Identifying myeloid sarcoma in rare locations is a diagnostic challenge and requires careful evaluation. The optimal management of extramedullary disease requires further investigation, but tissue biopsy and a personalized approach are crucial. Herein, we describe an unusual case of acute myeloid leukemia presenting with an isolated involvement of the temporal bone after a complete remission of systemic disease for more than a year. The clinical, radiological, and pathological features are discussed, highlighting the importance of considering differential diagnoses and appropriate management. A computed tomography scan reveals a complete homogenous left‐sided and partial right‐sided opacification of the mastoid cells and the middle ear with a relatively intact bony structure and no distinct masses.