Small cell carcinoma of the urinary bladder (SCCUB) is a rare, aggressive variant of neuroendocrine nonepithelial tumor. The best treatment results are achieved by combined cystectomy and chemotherapy. Same as in urothelial bladder cancer, a question remains about the urinary diversion in locally advanced disease. Studies on surgical treatment of patients with SCCUB most often do not clearly specify the type of urinary diversion performed, and we could not find information that orthotopic urinary diversion was performed in those patients. We present a 58-year-old patient with locally advanced SCCUB, treated by cystectomy and orthotopic urinary diversion (ileal neobladder-Hautmann) followed by six-cycle adjuvant chemotherapy. After 50-month follow-up, the patient showed no signs of relapse and the new bladder functioned perfectly well.