Case report: rare presentation of primary angiomyolipoma in the middle ear.

Background: Angiomyolipoma (AML) is a common, chronic disease usually localized in kidney and liver organs; but occurring frequently outside the kidney or liver.
Case Presentation: We describe an unusual case of a 62-year-old female patient with AML in the middle ear. She presented with left earache, tinnitus and hearing loss. Preoperative computed tomography and magnetic resonance imaging seemed to reveal a middle ear cholesteatoma. The patient underwent surgical resection of this lesion, and the AML was finally confirmed by histopathological examination. The patient was discharged on the 8th postoperative day and did not seek further treatment.
Conclusions: Extraperitoneal AML is rare and accurately identified by histopathology. The recommended management is surgery for AML in the middle ear.