Small cell carcinoma of the urinary bladder (SCCUB) is a rare and aggressive disease. To date, no standard treatment has been proposed due to the lack of prospective studies resulting from the rarity of this disease. Recently published studies of relatively large patient cohorts, however, have shed some light on the management of SCCUB patients. In this article, the authors review the epidemiology, pathogenesis, diagnosis and treatment (based on disease stage), and they then discuss the optimal therapeutic strategy for SCCUB patients, particularly for those with limited, locoregional disease. The authors conclude that multidisciplinary approaches are needed for the optimal management of this aggressive disease. The authors also discuss bladder-sparing approaches for SCCUB patients, compared to those for conventional bladder urothelial carcinoma patients.