A case of squamous cell carcinoma of the ureter in a 63-year-old female is reported. Right hydronephrosis was found by examination of duodenal ulcer. Ureteroscopy and biopsy revealed squamous cell carcinoma (SCC) of the lower ureter. Total nephroureterectomy with a bladder cuff was performed. Pathological diagnosis was SCC pT3 G2 INF beta pL1 pV0 pR0. Three cycles of chemotherapy were performed postoperatively with bleomycin, methotrexate and cisplatin. Fifty-three cases of primary ureteral squamous cell carcinoma from the Japanese literature are reviewed and characteristic clinical figures of the tumor are discussed.