A 48-year-old man presented complaining of lower abdominal pain, and was diagnosed with invasive bladder cancer. After neoadjuvant chemotherapy, cystoprostatourethrectomy and ileal conduit diversion were performed. At 53 years of age, bladder cancer recurred in the upper urinary tract and he underwent complete urinary tract extirpation. Histological examination revealed an urothelial carcinoma in an ileal conduit. Three years later, a local recurrence led to combined modality therapy including chemotherapy and radiotherapy. He is receiving maintenance chemotherapy for metastatic tumors. Urothelial carcinoma arising in an ileal conduit after radical cystectomy for bladder cancer is infrequently reported. To our knowledge, only 12 cases, including ours, have been reported.