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视神经胶质瘤（ONG）是一种好发于儿童和青少年且相对罕见的中枢神经系统肿瘤，主要病理类型为低级别的毛细胞型星形细胞瘤。其分为散发和1型神经纤维瘤病（NF-1）相关的ONG。由于ONG与视神经的紧密关系，在诊疗上存在其特殊性，其诊断主要依靠病史、症状和体征，以及磁共振成像和CT等影像检查。ONG应与神经鞘脑膜瘤、视神经炎、视神经转移瘤等疾病相鉴别。患者初诊时，可以暂行随诊观察治疗，密切关注患者视力、视野改变；当病情进展时，才需干预。化学药物治疗（化疗）是ONG的首选手段，近年来新发现的分子靶向治疗和抗血管内皮生长因子药物是ONG药物治疗的有力补充。当化疗不敏感或出现耐药时，可考虑放射治疗（放疗），但仅推荐对7岁以上患者进行。当患者视力损伤严重、突眼明显影响外观或发生暴露性角膜时，可考虑手术治疗。此外，由于NF-1患者存在肿瘤易患性，因此NF-1相关ONG患者的化疗方案和放疗时机与散发ONG患者存在不同，其化疗方案应考虑到药物引起继发性白血病的风险，放疗时机应在10岁以后。期待进一步的ONG临床研究能为今后的临床工作带来更多参考。“,”Optic nerve glioma (ONG) is a rare central nervous system tumor that occurs in children and adolescents. It’s main pathological type is low-grade pilocytic astrocytoma. It is divided into sporadic ONG and neurofibromatosis type 1 (NF-1) related ONG. Due to the close relationship between ONG and the optic nerve, there is its particularity in diagnosis and treatment. The diagnosis of ONG mainly relies on medical history, symptoms and signs, as well as imaging examinations such as MRI and CT. ONG should be differentiated from optic nerve sheath meningioma, optic neuritis, optic nerve metastasis and other diseases. In recent years, newly discovered molecular targeted therapy and anti-vascular endothelial growth factor drugs are a powerful supplement to ONG. When chemotherapy is not sensitive or resistant, radiotherapy can be considered, but it is only recommended for patients over 7 years of age. Surgery can be considered when the patient’s visual impairment is severe and the appearance of the eye is significantly affected. In addition, due to the susceptibility of NF-1 patients to tumors, the chemotherapy regimen should take into account the risk of secondary leukemia caused by the drug, and the timing of radiotherapy should be after the age of 10. We look forward to further ONG clinical research, which will bring more references for future clinical work.