Neurofibromatosis type 2 is an inherited disease. The main manifestation of the disease is the development of symmetric, non-malignant brain tumors in the region of the cranial nerve VIII usually as schwannoma. We report here a 20-year-old woman with primary intracranial malignant nerve sheath tumor located in the left cerebellopontine angle. Histologically, the tumor showed malignant spindle cells in fascicular pattern with focal S100 positivity on immunohistochemistry. A subtotal surgical resection was performed followed by adjuvant radiotherapy. This is the first case of primary intracranial malignant nerve sheath tumor associated with neurofibromatosis type 2 reported in the literature. The prognosis of this potentially aggressive neoplasm is poor.