A 75-year-old man visited our hospital presenting with gross hematuria. Cystoscopy revealed a bladder tumor with coagulated blood and necrotic tissue at the dome. We resected the bladder tumor transurethrally. Pathologically, the tumor was shown to be a transitional cell carcinoma with choriocarcinomatous differentiation, G3, > or = pT2. Immunohistochemical staining showed human chorionic gonadotropin (HCG)-positive tumor cells. Just after surgery, the serum HCG-beta concentration was less than 0.1 ng/ml. Total cystectomy and an ileal conduit operation were performed. The histological classification was TCC, G3, pTis. In later blood chemistry tests, HCG-beta elevation was observed. Pulmonary metastases appeared on a chest X-ray, and combination chemotherapy with cisplatin and etoposide was administered. Although the serum HCG-beta decreased with one course of chemotherapy, it increased immediately thereafter. The patient died of the disease about 8 months after the total cystectomy. Autopsy revealed multiple metastases in the lungs, liver, kidneys, stomach, pancreas, thyroid gland, adrenal glands, heart, jejunum, and vertebral body (Th10). Lymph node metastases in the pulmonary hilum, mediastinum, and right submaxillary gland were also found.