We present a 37-year-old female with a history of tuberous sclerosis. She developed flank pain, hypotension, and a sudden drop in hemoglobin levels which prompted a work-up. A computed tomography scan demonstrated enlarged heterogeneous kidneys and a large complex collection in the right kidney, likely hemorrhagic in nature. The findings were suspicious for bilateral angiomyolipomas with retroperitoneal hematoma. She was treated with bilateral transarterial embolization. She subsequently developed recurrent fever and was suspected of having emphysematous pyelonephritis, for which she underwent a right nephrectomy. The entire kidney was replaced by a tan bulging, lobular mass, with scattered tan-yellow nodules. Microscopic examination revealed an angiomyolipoma with an incidental oncocytoma. The association of renal angiomyolipoma and renal oncocytoma is rare and only 16 cases have been previously reported.
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