The lower pole of the kidney shows a 5 cm angiomyolipoma which closely resembles a renal cell carcinoma grossly. Sporadic angiomyolipomas are usually solitary and more frequently found in middle-aged women. They are often asymptomatic and usually discovered on imaging studies performed for unrelated conditions. Some cases present with massive retroperitoneal hemorrhage which may be fatal. Angiomyolipomas occurring in the setting of tuberous sclerosis are frequently bilateral, multiple, occur at younger age, and affect both genders.