Coexistence of renal cell carcinoma and angiomyolipoma in the same kidney is rare. A 64-year-old patient who had a hepatoma and who did not have tuberous sclerosis was admitted for examination of incidental massed found on abdominal sonography. A soft tissue mass involving segment 6 of right lobe of the liver and a huge retroperitoneal tumor on the right were found on computed tomography (CT). Findings on pathological examination of the liver biopsy specimen were consistent with poorly differentiated. Giant cell type, hepatocellular carcinoma. Findings on pathological examination of renal tissue were consistent with angiomyolipoma in the upper-lateral renal mass and with clear cell type, renal cell carcinoma in the upper-medial mass. After 12 months of regular follow-up, the patient died of hepatoma.