Renal cell carcinoma is rare in adolescents. Observation of a 16-year-old girl with a clear-cell carcinoma prompted us to review 372 published cases in adolescents. The tumor affects the sexes indifferently and has no side predominance. The incidence is higher in white races. The most frequent presenting sign is a palpable mass (52.7%). X-ray films typically show renal calcifications (25%). The most common histotype is the clear cell carcinoma (76.8%) and at diagnosis 59.5% of these tumors already extended beyond the kidney. The overall 5-year actuarial survival rate is 60.4%.