Small-cell carcinoma of the urinary bladder (SCCUB) accounts for less than 1% of all cancers arising in the urinary bladder. Current diagnosis and management of SCCUB are often patterned after small-cell lung cancer (SCLC). However, SCCUB therapy is different from that for SCLC. For example, many patients with SCCUB undergo local resection, which is rarely performed in SCLC. As in SCLC, platinum-etoposide combination chemotherapy is employed as the main systemic treatment option for SCCUB. Chemotherapy is usually combined with other therapeutic modalities, especially in patients whose disease is limited to the locoregional area. Owing to the rarity of this malignancy, no prospective study has been performed that establishes the efficacy and duration of chemotherapy or the relative efficacy of platinum-etoposide versus other chemotherapeutic regimens. This article provides a comprehensive review of the current status of SCCUB diagnosis and management, as well as some unique insights into this rare tumor.