A case of primary carcinoma of the remaining ureter is reported. A 70-year-old man presented with asymptomatic gross hematuria. Three years ago, he had received right nephrectomy for pyonephrosis. Although drip infusion pyelography (DIP) and cystoscopy showed no abnormal findings, computed tomography (CT) and retrograde ureterography demonstrated the irregular thickening of the right remaining ureteral wall. He underwent right ureterectomy with bladder cuff resection. Pathological examination revealed transitional cell carcinoma of the remaining ureter. He has been free of disease for 3 years.