We report a rare case of primary primitive neuroectodermal tumor (PNET) of the urinary bladder. An 85-year-old man with hormone refractory prostate cancer was presented with gross hematuria. Abdominal computed tomography showed a tumor occupying the right lateral portion and neck of the bladder wall, while a cystoscopic examination revealed a bleeding, sessile globular tumor with no papillary features. Transurethral resection of the bladder tumor was performed. Histopathological results showed a small round cell tumor forming rosettes, further positive immunoreactivities for some neural markers and MIC gene product (CD99), which indicated PNET of the bladder. Four months after surgery, PNET of the bladder recurred with rapid growth and the patient died of tumor. To our knowledge, there have been only 8 such cases reported.