BACKGROUND: The Endoscopic Endonasal Approach (EEA) has revolutionized the treatment landscape for optic pathway-hypothalamic gliomas (OPHGs) by providing precise visualization of the lesion neuroanatomic and minimizing brain manipulation. Despite the variable clinical trajectories of OPHGs, the optimal management neurosurgical strategy continues to be a subject of debate. METHODS: We present a case of an 8-year-old female with an OPHG who underwent EEA and performed a systematic review search in English and Spanish case reports from January 2007 (date of first reported case) to June 2024, focusing on OPHG treated with EEA. Databases such as PubMed, Lilacs, and Embase. SEARCH RESULTS: Our systematic review encompassed 30 cases of OPHGs treated with EEA. Most patients (86.6%) presented with visual disturbance, 43.3% with headaches, and 30% with endocrine disturbances. Gross-total resection (GTR) was achieved in 20%, subtotal in 40%, and near-total in 20%. Histologically, pilocytic astrocytoma predominated (73.3%), with varied clinical presentations highlighting the need for tailored management approaches. CONCLUSIONS: The EEA offers safe access to the optic pathway and hypothalamic region. Despite limitations in achieving GTR compared to other approaches, EEA's benefits in clinical improvement and neuroanatomical preservation make it a valuable option for select cases of OPHGs.