Primitive neuroectodermal tumor(PNET) is a rare tumor, comprising less than 1% of all soft tissue malignancies arising from the peripheral non-autonomic nervous system. Most of primitive neuroectodermal tumors were located in the extremities, thoraco-pulmonary region, and pelvic area and the urinary system is rarely involved. We report one case of primitive neuroectodermal tumor arising in the kidney, mimicking renal cell carcinoma on the CT scan.