Small cell carcinoma of the urinary bladder is a rare, often fatal, disease. Its presenting symptoms and gross morphology are similar to those of conventional urothelial carcinoma, whereas its prognosis is much poorer with frequent metastasis. Small cell carcinoma of the urinary bladder shares similar histology with its counterparts in other organs; however, its immunoreactivity to conventional neuroendocrine markers is low. Its diagnosis is thus considered permissible on morphologic grounds alone. Multimodal treatments are often employed, although no definite treatment algorithm has been established. For this extremely aggressive malignancy with an as-yet inconclusive etiology, further studies are needed to clarify its molecular pathogenesis to serve as a basis for diagnostic markers and therapeutic targets. The clinical, morphologic, immunoreactive, molecular, and therapeutic features of bladder small cell carcinoma are reviewed, including a detailed discussion on the utility of immunohistochemical markers.