A case of bilateral renal cell carcinoma in a 42-year-old polycystic kidney male is reported. He had been treated with hemodialysis for 22 years. An abnormal small mass was found in one of the left renal cystic lesions by screening ultrasonography and CT scan at the 19th year of the hemodialysis. Left radical nephrectomy was performed and the histological diagnosis was a renal cell carcinoma (RCC). There was no evidence of recurrence and metastasis, however, he presented with asymptomatic

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... ematuria two years after the operation. CT scan demonstrated the rapidly progressing right renal tumor and multiple para-aortic lymph node swelling. Right nephrectomy and lymphadenectomy were performed and pathological examination showed the advanced RCC with multiple lymph node metastasis. Eleven months after the second operation followed by interferon therapy, he died of multiorgan metastasis of the RCC. This is the first bilateral RCC case in polycystic kidney patient treated with hemodialysis in Japan.