Summary:Gliomas that arise from the brain stem typically have a poor prognosis. With improvement in neuroimaging and surgical technique, subclassification of brain stem glioma (BSG) is possible and provides prognostic significance. Those with a favorable prognosis account for a minority of BSGs. The majority of BSGs carry a poor prognosis, and survival has not dramatically changed with radiation therapy or chemotherapy. This review of BSGs will cover epidemiology, clinical presentation, prognostic factors, and treatment.