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. After ten years without recurrence, a relapse occurred. The diagnosis was recurrent clear cell sarcoma of the kidney. The course in our case is uncommon because clear cell sarcoma of the kidney is a rare and aggressive pediatric neoplasm, usually characterized by a predilection for bone metastases, and a mid survival rate of 30 months without treatment.
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