Abstract Purpose To investigate the clinical features of pediatric Xp11.2 translocation renal cell carcinoma (RCC). Methods A retrospective review of 22 cases over 35years. Results Xp11.2 translocation RCCs were identified in 13 boys and nine girls with a median age of 10.5years (range: 2.5-16years). RCC presented with: hematuria in 17, abdominal mass in one, abdominal masses with hematuria in two, abdominal pain with hematuria in one, and as an incidental finding in one patient. Ten patients were classified stage I, 10 were stage III, and two were stage IV. Of the 10 patients with stage I RCCs, three patients with tumor measuring less than 7-cm had nephron-sparing surgery (NSS) and 17 patients underwent simple nephrectomy. A 15-cm tumor was incompletely removed in one patient and another patient with a 25cm×18cm×15cm tumor had gross residual. Of the 15 patients followed-up between 6months and 35years, 13 were still living and two had died after surgery. Conclusions Xp11.2 translocation RCC is the predominant form of pediatric RCC, associated with advanced stage at presentation. Nephrectomy is the usual treatment for RCC but NSS is an option for patients with tumors measuring<7- cm. Patients with N+M0 maintained a favorable prognosis following surgery alone.