Abstract
Renal tumours vary from a benign to highly malignant histology. Here we present a 54 yrs male with a heterogeneous large encapsulated cystic lesion in the right kidney upper pole showing areas of solid component and necrosis with no evidence of metastasis or paraneoplastic features. Post Right Radical Nephrectomy IHC, showed positive reaction for BCL-2, CD99 and vimentin, suggesting Primary Renal Synovial Sarcoma. The patient was treated with ifosfamide based chemotherapy in view of unpredictable prognosis. We present this case for its rarity and good response to treatment and as a suggestion for differential diagnosis of Renal Cell Carcinoma.