Abstract
Primary renal synovial sarcoma (SS) is an exceptionally rare malignancy, often indistinguishable from other renal tumors on imaging. We report a case of a 67-year-old male presenting with hematuria and a renal mass, later diagnosed as monophasic SS with synchronous pulmonary metastases. Diagnosis was confirmed through histopathology, immunohistochemistry, and cytogenetics. The patient underwent cytoreductive nephrectomy followed by adjuvant chemotherapy. This case highlights the diagnostic challenges and underscores the importance of a multidisciplinary approach in managing metastatic renal SS.