Myeloid sarcoma (MS) is a proliferation of immature myeloid cells that occurs extramedullary, often accompanying acute myeloid leukemia (AML). It typically presents in areas such as lymph nodes, skin, and the head and neck regions. The emergence of MS within the temporal bone is rare, and it can manifest through nonspecific symptoms. In this paper, we reported a case of A 47-year-old female presented with right-sided otalgia, otorrhea, tinnitus, reduced hearing, and ear fullness, and developed facial asymmetry. She was initially diagnosed with necrotizing otitis externa and was treated accordingly. Further investigations were conducted, leading to the diagnosis of MS. She began treatment with induction chemotherapy followed by consolidation therapy. And we reviewed the literature and included 14 MS cases with the same anatomical localization, discussing the primary presentation, immunohistochemistry, and treatment approaches of these patients. In conclusion, temporal bone MS should be considered in patients with a history of AML presenting with nonspecific symptoms. Diagnostic modalities for MS include computed tomography and magnetic resonance imaging scans. Systemic chemotherapy remains the preferred treatment approach. The complexity and diversity of temporal bone MS presentations necessitate further comprehensive research to enhance understanding and improve management strategies for this rare condition.