A case of renal cell carcinoma (RCC) in a teenage patient is reported. A 13-year-old girl visited our hospital with the complaint of dyspnea and right abdominal mass. CT scan and chest X-P revealed a right renal tumor with multiple lung metastasis. Wilms' tumor was suspected and a combination chemotherapy consisting of actinomycin D and vincristin was performed. Since the tumor was insensitive to these agents, open biopsy was done. The pathological findings showed clear cell renal cell carcinoma. Despite the administration of alpha interferon, 5-FU and cimetidine, the disease was progressive and the patient died 5 months after diagnosis. It is important to consider the possibility of renal cell carcinoma in teenage patients with a renal mass, since more than 40% of the renal tumors in this age group are renal cell carcinomas.