Primary small cell carcinoma of the renal pelvis is a rare and aggressive disease; reportedly, a mean survival is only 8 months. A 78 year-old woman with chronic kidney disease was referred to our hospital complaining of asymptomatic gross hematoturia. On imaging studies and voided urine cytology, diagnosis of right renal pelvic cancer (cT2N0M0) was made. She underwent total nephroureterectomy. Pathological diagnosis was small cell carcinoma, infiltrating into the renal parenchyma, with lymphovascular invasion. Post-operatively, hemodialysis was introduced. Five months after the operation, new lesions developed in the right adrenal gland, aortocaval lymph nodes and subcutaneous layer of the right back. The subcutaneous mass was surgically removed and low-dose chemoradiotherapy (sigma 45 Gy/25 Fr/32 d + cisplatin 10 mg/d for 2 d x 2) was given to the other lesions. Although the lesions regressed to CR, new small masses emerged in the muscle layers of the right flank 14 months after total nephroureterectomy. Low-dose chemoradiotherapy (sigma 40 Gy/20 Fr/29 d + cisplatin 10 mg/d for 2 d x 2) to these lesions successfully brought CR. She is alive without any evidence of disease at 3 years after total nephroureterectomy.