We report the case of a kidney tumor occurring in a thirteen year-old
girl. This tumor was first diagnosed and treated as a benign one. Afte
r ten years without recurrence, a relapse occurred. The diagnosis was
recurrent clear cell sar coma of the kidney. The course in our case is
uncommon because clear cell sarcoma of the kidney is a rare and aggre
ssive pediatric neoplasm, usually characterized by a predilection Sor
bone metastases, and a min survival rare of 30 months without treatmen
t.