Primitive neuroectodermal tumor (PNET) of kidney is an extremely rare renal neoplasm with only about fifty reported cases in literature. Presumably,of neural crest origin, these tumors behave aggressively and carry a poor prognosis. We report a case of 22-year old female patient complaining of left loin pain with recurrent hematuria for last 3 months. On clinical examination, the abdomen was soft and no palpable mass was felt. She underwent ultrasonography and com puted tomography, which revealed a left renal mass. A left radical nephrectomy was performed. Histopathological examination of the nephrectomy specimen showed features of primitive neu roectodermal tumor arising from left kidney, which was confirmed by immunohistochemistry (IHC). The patient was treated with post-nephrectomy chemotherapy and was symptom-free at six-month follow-up.