Renal cell carcinoma (RCC) is the most common tumor of the kidney. Angiomyolipoma is frequently present in the patient who has the stigma of tuberous sclerosis. We reported a case of a 39-year-old female, without the stigma of tuberous sclerosis, who was admitted on account of a firm, huge, fixed and palpable mass over the left flank region for one week. After radical nephrectomy, the pathological report was renal cell carcinoma, granular cell type, associated with angiomyolipoma. The literature concerning this unusual case is reviewed.