Malignant peripheral nerve sheath tumors are highly aggressive tumors characterized by rapid growth with infiltration of surrounding tissue and hematogenous metastases. We described a 32-old male patient with malignant peripheral nerve sheath tumor of the head and neck. Surgery was the chosen method of treatment by the dissecting the mass. Even chemotherapy were administered after surgery, local recurrence occurred in the second month of the follow-up period. Then the patient underwent radical neck dissection under general anesthesia. Close follow-up was made, at first year disease had no recurrence. We reported this case and reviewed the literature regarding malignant peripheral nerve sheath tumor by reason of this case.