Primary tumor originated from ureteral stump following nephrectomy for benign disease is extremely rare. A 79-year-old male was referred to our department for asymptomatic macroscopic hematuria. He had undergone left simple nephrectomy for renal tuberculosis 52 years ago. Cystoscopic examination revealed a ureteral tumor on the residual ureteral orifice. Under the diagnosis of left ureteral stump tumor, which was subsequently enhanced on computer tomographic scan and magnetic resonance imaging, he received partial cystectomy and excision of the left ureteral stump. The histological examination revealed grade 2 to 3 urothelial carcinoma with muscle invasion (pT2). He received no adjuvant chemotherapy. He is now alive and free from recurrence 2 months post-operatively. This is the 21st case reported in the Japanese literature.