Objective: To evaluate the clinical features and outcome of children with renal cell carcinoma (RCC). Patients and Methods: Eleven patients with RCC who were diagnosed between 1972 and 2004 were retrospectively analyzed. Clinical features, histopathology, treatment regimens and outcomes of the patients were evaluated. Results: The male/female ratio was 3: 8, with a median age of 10 years. The stage distribution was as follows: 3 patients in stage I, 1 patient in stage II, 3 patients in stage IIIb, and 4 patients in stage IV. Five of 7 patients with stage II-IV received an actinomycin D-based regimen, one received a cisplatin-based regimen, and the other was given 5-fluorouracil (5-FU). In the last patient, interferon-alpha was given in combination with 5-FU; 1 of the patients on the actinomycin D regimen received interferon-alpha as well. All of the stage I patients are alive without disease. Three patients with stage IIIb, stage IV and stage II disease are alive without disease 8, 14 and 26 years after their diagnosis, respectively. The other stage IV and stage IIIb patients died of the disease. Conclusion: Nephroureterectomy is the main treatment modality, and it is sufficient for stage I patients. For patients with stage II-IV RCC, interferon-alpha and/or actinomycin D-based chemotherapy is the treatment of choice. Copyright (c) 2007 S. Karger AG, Basel.