The peripheral malignant neuroepithelioma is a rare neuroectodermal tumor arising within the peripheral nervous system. It usually occur in the lower extremities and often occur in shoulder and upper arms. It occurs in patient at any age. The children, less than the five years old of age, the tumor must be differentiated from the metastatic neuroblastoma; The tumor, in adolescent and adults, must be distinguished from other malignant round-cell tumors. The prognosis is poor and the aggressive, combined surgical and chemotherapeutic modalities are needed to treat this tumor, so a prompt and accurate diagnosis is necessary.