Malignant peripheral nerve sheath tumor (MPNST) is an uncommon neoplasm that rarely involves the head and neck region. We report the case of a 66-year-old man who presented with symptoms compatible with uncinate seizures. Imaging studies detected a left infratemporal fossa tumor with bone tissue destruction and extension into the intracranial compartment, which exerted a mass effect on the temporal lobe. Histologic studies identified the tumor as an MPNST. Complete removal of the lesion was not possible, so the patient was treated with adjuvant radiotherapy. He died 6 months later. This rare case of MPNST with intracranial involvement illustrates the dismal prognosis for patients with such a lesion. Survival is limited because of the difficulty of performing radical surgery with free margins in such a location.