Introduction: Clear cell sarcoma of the kidney (CCSK) is a rare renal tumor which accounts for 3% of renal tumors. The tumor derives its name from the clear cytoplasm of the predominant cell type. Herein, we report a case of renal mass for which a radical nephrectomy was done and histologically proved to be CCSK. Case Report: A 34 year female presented with hematuria, dysuria, right flank pain and fever. Investigations revealed locally advanced right renal mass with right renal vein thrombosis. She underwent right radical nephrectomy followed by radiotherapy. Histopathology showed 10x11x11 cm of clear cell sarcoma tumor in upper and mid pole with multiple metastatic nodules in renal sinuses and in the lower pole. Conclusion: Clear cell sarcoma of the kidney is one of the rare renal tumor, we have added this case to the few cases published in the literature.