Optic pathway gliomas (OPG) are the most common primary tumors of the visual pathways, and constitute 1% of all brain tumors and 5% of all brain tumors in children. Among Neurofibromatosis type 1 patients (a hereditary genetic disorder which is characterized by higher incidence of tumors from a neurocutaneous origin) it is the most frequent tumor and it constitutes between 15 to 20 percent of all nervous system tumors. These tumors are stable most of the time and remain indolent for many years after diagnosis, especially in patients suffering from Neurofibromatosis type 1. However, amongst some of the patients suffering from OPG, these tumors develop progressive characteristics and can cause visual disturbances, endocrine dysfunction, blindness and even death. Patients with aggressive tumors will need treatment, which can be either surgery, chemotherapy or radiation therapy. Today, the treating physicians face substantial difficulty in estimating the course the tumor will take, choosing the right candidates for oncological treatment and the type of therapy most suited to the case, due to lack of reliable information in the relevant literature. This article characterizes the tumors, presents updates from recent literature, as well as recommendations for treatment and follow-up.