A 77-year-old man visited our hospital with a chief complaint of asymptomatic gross hematuria. He was diagnosed with right renal pelvic tumor (7 cm) involving right renal hilar and inter-aortocaval lymph node metastases by radiological evaluation, and cytologic examination of urine indicated small cell carcinoma. After neoadjuvant chemotherapy with gemcitabine and cisplatin, right nephroureterctomy with bladder cuff, and right renal hilar and inter-aortocaval lymph node dissection was performed. Histological examination of the specimen revealed a small cell carcinoma of the renal pelvis (ypT3N2). After the operation, adjuvant chemotherapy with etopside and carboplatin was administered in combination with radiation therapy. At 5 months after the operation, there has been no evidence of recurrence. To our knowledge, this is the 38th report of a small cell carcinoma originating from the kidney in the literature.