Abstract: Objective To summarize the clinical characteristics of non-arteritic anterior ischemic optic neuropathy (NAION) associated with congenital occupying lesions in optic disc. Design Retrospective case series. Participants A total of 9 rare cases (11 eyes) with NAION associated with congenital optic disc occupying lesions, in Beijing Tongren Hospital between January 2010 and June 2019 were collected. Methods Ophthalmic and cranial neuroimaging data of patients were reviewed. All patients were followed up for at least 6-mouths. Main Outcome Measures Age, the effected single/both eyes, visual field defect, neuroradiology data. Results There were 4 cases of melanocytoma, 3 cases of drusen and 2 cases of myelinated nerve fiber which were associated with NAION in this study. The median age of disease occurred were 39 years old (range: 26-65 years). Involvement of monocular congenital optic disc occupying lesion was happened in all patients of melanocytoma and myelinated nerve fiber which were also suffered from unilateral NAION at the same eye. However, the opposite situation occurred in 3 patients of drusen, whose lesions were all bilateral but 2 patients were suffered from NAION in both eyes successively. The best corrective visual acuity variation of 11 eyes was 0.05 to 1.0 and automated static perimetry was presented with horizonal inferior depression and concentric defection in 4 patients. The deterioration of visual function and the reoccurrence of disease were never happened in all patients on last follow-up. Conclusions Some congenital optic disc occupying lesions could suffer severe visual dysfunction on account of the occurrence of NAION. （Ophthalmol CHN, 2020, 29: 141-146）

Key words: optic disc, melanocytoma, drusen, myelinated nerve fiber, non-arteritic anterior ischemic optic neuropathy