Angiomyolipoma is a well described but relatively uncommon benign renal neoplasm composed of varying admixtures of mature adipose tissue, smooth muscle, and thick-walled blood vessels. The incidence of angiomyolipoma is about 0.3% overall. It frequently occurs in patients with tuberous sclerosis. Even more uncommon is the simultaneous occurrence of angiomyolipoma and renal cell cancer in the same kidney in a patient without tuberous sclerosis.