Renal cell carcinoma (RCC) is a relatively uncommon tumor in childhood. Its biologic behavior and prognostic factors have rarely been documented. We report treatment and survival of 4 children (aged 8, 9, 11, and 14 years) who had RCC, along with a review of the literature to analyze the frequency of major symptoms, clinical stage, and prognostic factors based on 130 published cases of RCC in individuals younger than 20 years of age. Two of our cases had renal tumors detected by ultrasound screening, and all 4 cases were followed for a considerable length of time and were alive and free of disease after treatment. An analysis of these 130 published cases of pediatric RCC showed tumor staging and cell type to be the factors that affected patient survival. Tumors composed of granular cells or mixed cells, or at advanced stages, had a poor prognosis. Age, sex, tumor size, symptom duration, and cellular pattern were not related to patient prognosis. Children older than 10 years of age, who have an abdominal mass, flank pain, and/or hematuria should alert clinicians to consider the possibility of RCC. The importance of early diagnosis of renal tumors, using ultrasound as a tool of screening, is emphasized, since surgical treatment leads to a favorable prognosis only in the early stage of RCC.