We aim to review the imaging appearance of peripheral nerve sheath tumors (PNSTs) of head and neck according to updated fourth edition of World Health Organization classification. Peripheral nerve sheath tumor can be sporadic or associated with neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Schwannoma is the most common benign PNST that can be intracranial or extracranial and appears heterogeneous reflecting its histologic composition. Melanotic schwannoma is a different entity with high prediction of malignancy; it shows hypointense signal on T2-weighted image. Neurofibroma can present by localized, plexiform, or diffuse lesion. It usually appears homogeneous or shows a characteristic target sign. Perineurioma can be intraneural seen with the nerve fiber or extraneural appearing as a mass. Solitary circumscribed neuroma and neurothekeoma commonly present as dermal lesions. Nerve sheath myxoma may exhibit high signal on T1 weighted image. Benign triton tumors can be central, aggressive lesion, or peripheral nonaggressive lesion. Granular cell tumor shows hypointense signal on T2 weighted image. Neuroglial heterotopia most commonly occurs in the nasal cavity. Ectopic meningioma arises from ectopic arachnoid cells in the neck. In hybrid PNST, combined histological features of benign PNST occur in the same lesion. Malignant PNSTs are rare with an aggressive pattern. Computed tomography and magnetic resonance imaging are complementary studies to determine the location and extent of the tumor. Advanced magnetic resonance sequences, namely, diffusion-weighted imaging and dynamic contrast enhancement, can help in differentiation of benign from malignant PNST.
