We reported a case of ileal segment adenocarcinoma arising in the augmented bladder 40 years after the operation. The patient was a 57-year-old man who underwent ileocystoplasty (Goodwin method) for contracted bladder due to tubercurosis in 1962. He was referred to our clinic for examination of gross hematuria. Cystoscopy revealed a tumor on the ileal segment of the augmented bladder. He underwent resection of the ileal segment and ureterocutaneostomy. The pathological diagnosis was poorly differentiated adenocarcinoma in the ileal segment. He died of the disease 6 months after the operation.