Primitive neuroectodermal tumor (PNET) is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS). PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET). This tumor which expresses MIC-2 gene (CD99) seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.