Small cell carcinoma of the bladder (SCCB) is extremely rare. In this paper, we present a case of metastatic SCCB managed by chemotherapy and we would provide a brief review of the epidemiology, clinical features, diagnosis, pathologic features, staging, treatment, and prognosis of SCCB. A 52-year-old man was admitted with signs and symptoms suggestive of a bladder cancer. Computed tomography of the pelvis and abdomen showed a large tumor at the right bladder wall, measuring 10 cm in diameter, and a multinodular liver disease. Diagnosis of small cell carcinoma was established from the histological study of the transurethral resection of the bladder tumor. The patient received 12 cycles of platinum-based chemotherapy with a good partial response of bladder tumor and liver metastasis. The patient is still alive, 18 months after diagnosis.