A 69 year-old woman visited our hospital with a chief complaint of macrohematuria in April 2004. She was diagnosed with bladder tumor. She underwent transurethral resection ofbladdar tumor three times and right partial ureterectomy for ureteral tumor following primary bladder carcinoma. All pathological findings demonstrated that the tumor was urothelial carcinoma (UC), G1-G2, pT1 including right ureteral tumor. In spite of intravesical instillation of BCG, recurrent invasive bladder tumor was found in June 2006. In July 2006, we performed total cystectomy and construction of ileal conduit. Surgical specimen revealed small cell carcinoma and immunohistochemical staining with NSE and synaptophysin was positive. On day 67 after operation, the patient died of multiple metastases to liver and bone.