Primitive neuroectodermal tumor (PNET) of the kidney is an extremely rare renal neoplasm with only about 50 reported cases in the literature. These tumors behave aggressively and carry a poor prognosis. A 22 years female patient presented with right lumber and right hypochondrium lump of 4 months duration. Commutated tomography revealed large right renal mass with renal vein and inferior vena cava (IVC) thrombus. Magnetic resonance imaging abdomen demonstrated the extension of tumor thrombus up to the junction of hepatic vein and IVC. Preoperative percutaneous needle biopsy was performed. Histopathology demonstrated small round to oval cells with scanty cytoplasm and cells are arranged in clusters. Immunohistochemical staining demonstrated a highly specific cluster of differentiation 99, confirming the diagnosis of a PNET.