Primitive neuroectodermal tumors of the kidney are rare tumors representing about 1% of all sarcomas. Extraskeletal primitive neuroectodermal tumors may be found in the genitourinary tract, the testes, ovaries, uterus, or pancreas. Renal primitive neuroec- todermal tumors are frequently aggressive and almost 30% of all newly diagnosed cases present with distant metastases. Surgical intervention, intensive chemotherapeutic drugs and radiation therapy are the best choices for management. We discuss the case of a 22-year-old male who presented with a left renal mass. He underwent nephrectomy followed by histopathological examination. Microscopic and immunohistochemical examination revealed a primitive neuroectodermal tumor.