Renal angiomyolipoma is a distinctive benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex. A 45-year-old woman was admitted with history offlank pain and vomiting. There were no signs suggestive of tuberous sclerosis either in the patient or her family. At operation, she had a left renal mass with nephrolithiasis and hydronephrosis. Histopathology revealed epithelioid angiomyolipoma of the left kidney with chronic pyelonephritis. Immunohistochemistry confirmed the diagnosis of angiomyolipoma. This case is presented to highlight the epithelioid variant of angiomyolipoma which may behave in an aggressive manner.
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