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Renal synovial sarcoma is a recently reported neoplasm rarely seen. We report a case of primary renal synovial sarcoma. The signs and symptoms are similar to any primary renal tumour diagnosis is clinically difficult through general survey or multiple imaging modalities and requires specific molecular and genetic testing. Surgical resection and ifosfamide-based chemotherapy are the mainstay of management. The prognosis can be poor. Physicians should be aware that synovial sarcoma is one of the possibilities of malignancy in the kidney.
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