Neurofibromatosis 1 is associated with an increased risk for the development of benign and malignant tumors involving neural and non-neural tissues. Children as well as adults with neurofibromatosis 1 are affected. Central nervous system neoplasms represent a significant portion of these malignancies and appear primarily in children less than 10 years of age. Optic pathway gliomas and brainstem gliomas are the most common intracranial neoplasms found in neurofibromatosis 1, although there also is an increased incidence of other brain tumors in this population. The majority of these intracranial neoplasms are benign pilocytic astrocytomas, which may behave in a less aggressive manner than histologically identical tumors in non-neurofibromatosis 1 patients. Owing to the indolent nature of these tumors, conservative management with close follow-up is recommended. When intervention is required, conventional treatment with surgery, radiation, or chemotherapy has been used with variable results. The current challenge lies in understanding the pathogenesis of gliomas in neurofibromatosis 1, which may lead to the development of biologically directed therapies with less associated morbidity and mortality for neurofibromatosis 1 as well as non-neurofibromatosis 1 children.