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Carcinosarcoma of the urinary bladder is a rare neoplasm that is composed of malignant epithelial and mesenchymal components. Fewer than 80 cases have been reported worldwide, usually as case reports or a small series. A series of 221 cases using the Surveillance, Epidemiology and End Results (SEER) Program database has been reported recently. We present one case of carcinosarcoma that was managed by neoadjuvant chemotherapy and that achieved a complete pathological response. A 57-year-old male patient presented with a 2-month history of hematuria. Ultrasonography revealed a 4 cm mass in the bladder dome. A transurethral resection of the bladder tumor (TURBT) revealed a carcinosarcoma. The patient underwent subsequent neoadjuvant chemotherapy and radiotherapy. The pathology specimen examination revealed no residual tumor. The patient had no evidence of recurrence after 20 months. This patient is the second reported case of carcinosarcoma of the urinary bladder that achieved a complete pathological response after neoadjuvant chemotherapy. [ABSTRACT FROM AUTHOR]