An 82-year-old woman visited our hospital with right flank pain. Computed tomography showed a right ureteral tumor. Urine cytology was class IV and cystoscopy demonstrated no obvious lesion. We performed right total nephroureterectomy and histopathological diagnosis was small cell carcinoma of the ureter. The serum ProGRP was slightly elevated postoperatively. Positron emission tomography showed a distant metastasis to the third lumber vertebra. She received two courses of chemotherapy (cisplatin and etoposide) and radiation therapy (8 Gy), but the distant metastasis was progressive. She died 6 months postoperatively.