Clear cell sarcoma of the kidney is the most frequently misdiagnosed renal tumor in children. We report the case of a 6-year-old boy with clear cell sarcoma of the kidney with an unusual presentation, including a primary tumor of the left kidney with metastasis in the homolateral psoas muscle. The renal tumor was revealed by abdominal mass without hematuria. In a review of the literature. Clear cell sarcoma of the kidney is most commonly associated with bone and lung metastases. Muscular metastasis at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of clear cell sarcoma of the kidney. This also illustrates clear cell sarcoma of the kidney's ability to metastasize to other sites including the muscular. These tumors present a diagnostic challenge for the radiologist who should be aware of this entity to differentiate it from other renal tumors which are more frequent at this age. We aim to report the clinical, radiological features, and pathological presentation of this entity.
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