OBJECTIVE: To report a case of renal cell carcinoma associated with renal angiomyolipoma and renal cortical adenomaand discuss the possibility of a hereditary etiology.METHODS: A 64-year-old patient with a tumor in the rightkidney underwent right renal nephrectomy. The pathological findingsrevealed a clear cell renal carcinoma, two cortical angiomyolipomaand a renal cortical adenoma. The literature on these three coincidentrenal tumors is reviewed.RESULTS: The association of these three renal tumors is rareand is usually seen in patients with tuberous sclerosis.CONCLUSIONS: Renal angiomyolipoma is a benign tumorfrequently associated with Bourneville’s disease or tuberous sclerosis,a disorder of autosomal dominant transmission. Familial evidenceof renal cell carcinoma has been previously reported, as in vonHippel-Lindau disease. It has been suggested that heredity may playan important role in the association of renal cell carcinoma andother renal tumors.