Abstract: We report a case of primary small cell carcinoma of the ureter. These tumors are very rare, locally aggressive and with poor prognosis. A 77-year-old man presented with left flank pain and microhematuria. Following diagnosis of ureteral carcinoma, nephroureterectomy was carried out, and adjuvant chemotherapy and radiotherapy were given. The patient had no evidence of recurrence or metastasis, 13 months postoperatively. We provide a bibliographic review and comment on its clinical and pathologic characteristics, diagnostic methods and latest modifications in therapy.