Objective: To characterize a clinical triad of symptoms associated with myeloid sarcomas of the temporal bone via a review of all previously reported cases. Methods: Case report and Ovid MEDLINE database literature review. Results: A literature search revealed that a clinical triad of hearing loss, otalgia, and facial nerve weakness are commonly associated with this rare presentation of myeloid sarcoma in the temporal bone. 44% (18/41) of patients presented with all three symptoms, while 76% (31/41) presented with at least two. The presence of t(8;21) was reported in nine patients with myeloid sarcomas of the temporal bone.Conclusions: Although myeloid sarcomas are exceedingly rare, it is necessary to consider them as part of the differential diagnosis for patients who might present with middle ear and mastoid opacification on computed tomography (CT) scan, hearing loss, otalgia, and facial nerve palsy. Physicians should maintain a high degree of suspicion in patients with a history of acute myelogenous leukemia (AML), especially if previous cytogenetic analysis revealed a t(8;21).