- Primitive neuroectodermal tumor (PNET) arises from primitive cells of the neural crest. A common cytogenetic abnormality is t (11, 22)(q24;12). Cases with a renal origin are rare entities, but have been increasingly reported in recent years. There is no consensus on the standard of care treatment for metastatic PNET of the kidney because of its rarity. Primary treatment consists of multiagent chemotherapy. The treatment options for recurrent or refractory disease include: a clinical trial, ifosfamide with mesna and etoposide; ICE (ifosfamide, carboplatin and etoposide); gemcitabine with docetaxel; topotecan with cyclophosphamide; and irinotecan and temozolomide. We present a case of primary renal PNET and discuss treatment options for metastatic disease.