This report describes a very rare case of lipoblastoma of a kidney affecting a small child. A 2-year-old boy was admitted for a palpable mass in the right abdominal quadrant. A preoperative examination revealed a tumor of uncertain origin. A simple nephrectomy was performed. Postoperative histologic examination confirmed lipoblastoma of the kidney. No other therapy was necessary. The child is well, without any sign of the disease 9 years after the tumor removal.