Abstract
Peripheral primary neuroectodermal tumors (PNET) are uncommon tumors, with an overall incidence of 1% of all sarcomas. Renal PNET is extremely rare. It is an aggressive tumor, with a high incidence of local recurrence and early metastases. Radiological diagnosis can be challenging as it is often difficult to differentiate it from other primary renal neoplasms. Imaging features include a large heterogeneous mass with central low density areas due to necrosis. Intra tumor hemorrhage and calcification are rare. We present a rare case of a large PNET mimicking renal cell carcinoma in a 51 year old female that required arterial embolization and nephrectomy.