Renal cell carcinoma is a rare renal tumor in the pediatric population with an incidence of 0.1-0.3%. We report on the first case of bilateral renal clear cell carcinoma that was treated by partial nephrectomies. We performed a complete genetic evaluation to exclude a family inheritance and we looked at the deletions associated with the possible histologic subtypes of renal cell carcinoma. The patient had no evidence of recurrence after 80-month follow-up and her renal function remained normal. © 2012 Elsevier Inc.