AIM: To describe the clinical features of acute myopic onset of optic neuropathy and observe the effects of retrobulbar and systemic glucocorticoid therapy in a real-world setting. METHODS: A retrospective observational case series included 18 patients with a clinical diagnosis of acute onset of myopic optic neuropathy in a real-world setting. While the patients were using retrobulbar and systemic glucocorticoid therapy, various imaging examination data were analysed, and the clinical features of myopic optic neuropathy were summarized for 6mo to 2y. RESULTS: The included group of patients with acute onset of myopic optic neuropathy consisted mostly of females (n=11). The visual field (VF) showed abnormalities in bilateral eyes, including the spread of physiological blind spots, central and paracentral dark spots, and centripetal peripheral VF reduction; but central vision with no subjective changes. The visual evoked potential (VEP) was abnormal in all eyes with vision loss. The best corrected visual acuity (BCVA) was improved from 1.04±0.63 to 0.47±0.57 (logMAR) after glucocorticoid treatment (P<0.05). In patients with a short course (within 1wk), recovery was fast and achieved the same BCVA as recorded before the onset within 6d. However, in patients with the long course (1 to 2wk), recovery was slow and did not achieve the BCVA recorded before the onset within 10d. The changes of intraocular pressure (IOP) were not obvious before and after treatment (18.68±5.30 vs 19.55±5.34 mm Hg, P>0.05). There was no recurrence during long-term follow-up observation. CONCLUSION: The acute onset of myopic optic neuropathy is characterized by BCVA and VF abnormalities in bilateral eyes. Retrobulbar and systemic glucocorticoid therapy is effective.