Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with an incidence of about 1:2500 to 1:3000. It is caused by a germline inactivating mutation of the NF1 gene on chromosome 17. Patients with NF1 are at increased risk of developing a variety of tumors of the peripheral and central nervous system, including neurofibromas, plexiform neurofibromas, malignant peripheral nerve sheath tumors, and low-grade gliomas of the optic nerves and other cerebral structures. Rarely, they develop high-grade gliomas. Although they are rare, these hereditary tumor syndromes involving the nervous system must be recognized in patients and their families, as early diagnosis may alter management and ultimately improve outcome. Additional insight into the molecular mechanisms causing these syndromes and their relationship with the clinical features will allow the development and implementation of screening and prevention strategies for these diseases. Management of these lesions is difficult and requires specific skills and the collaborative work of neurosurgeons, radiation therapists, neurologists, and oncologists. Ideally, patients should be managed in comprehensive centers with specific expertise in the management of patients with NF1. This review describes current and developing therapies for managing the neuro-oncologic manifestations of NF1.