Primary small cell carcinoma of urinary bladder is a rare but aggressive disease with poor prognosis and a high mortality rate. It accounts for less than 1 % of all the primary cancers seen in the urinary bladder. Diagnosis and management of this entity poses a challenge to the clinician due to the lack of a standardized protocol for its treatment. Herein we discuss primary small cell carcinoma of the urinary bladder in its entirety.