Optic pathway gliomas (OPGs) are a rare type of low-grade glioma in children and account for 1%~5% of brain tumor cases in children, and OPGs associated with neurofibromatosis-1 account for 30%~50% of all cases of OPGs. This article reviews the latest research advances in the imaging examination, diagnostic methods (pathological and molecular pathological diagnosis and ophthalmic examination techniques), and treatment methods (surgery, chemotherapy, and radiotherapy) for OPGs in children, so as to provide new perspectives for clinicians and scientific research.