The purpose of this review is to discuss the contemporary diagnosis and treatment options for nerve sheath tumors. Common nerve sheath tumors include schwannomas, neurofibromas and malignant nerve sheath tumors. Nerve sheath tumors can be associated with genetic disorders such as neurofibromatosis and schwannomatosis. There is great variation in terms of tumor location, clinical presentation and treatment strategy. Magnetic resonance imaging is the diagnostic study of choice for nerve sheath tumors and surgery has become the mainstay of treatment for most symptomatic lesions. Progress in microsurgical techniques has resulted in significant improvement in surgical outcome, with preservation of neurologic function. Complete resection of benign nerve sheath tumors is the goal of surgical intervention and this results in cure. In contrast, malignant nerve sheath tumors remain a major challenge with poor outcomes overall. Progress in multidisciplinary research may ultimately lead to novel therapeutic strategies.