Clear Cell Sarcoma of the Kidney (CCSK) is a rare malignant childhood tumor with frequent metastasis to the bone. We report a case of right sided in a 5 month-old girl. A radical nephrectomy was performed. It was clinical stage III with renal capsular invasion and lymph node metastasis by the classification of NWTS-5. Histologic examination revealed the classic pattern of CCSK. Postoperative adjuvant chemotherapy with doxorubicin and radiotheraphy were applied.