A case of renal cell carcinoma and bladder carcinoma associated with von Hippel-Lindau disease is reported. A 31-year-old female was referred to the Department of Urology for further examination of right renal mass which was incidentally found on abdominal computed tomography (CT). The patient was operated on spinal hemangioma in May 19 and July 8, 1975, on cerebellar hemangioblastoma in July, 1976 and June 10, 1981 and on cerebellar cyst in June 20, 1988. Angiography revealed three hypervascular renal tumors in the right kidney. Cystoscopy revealed a papillary bladder tumor (TCC Grade 1). Transurethral resection of bladder carcinoma was performed on July 28, 1988. Right radical nephrectomy and lymphadenectomy were performed on August 2, 1988. Histopathologically, the tumor was renal cell carcinoma of clear cell type (Grade 1). Postoperative course was uneventful and the residual kidney is being followed up in the outpatient clinic
