Optic neuritis (ON) is the most common cause of acute optic neuropathy in patients younger than 50 years of age and is most frequently idiopathic or associated with multiple sclerosis. However, the discovery of aquaporin-4 immunoglobulin G (IgG) and myelin oligodendrocyte glycoprotein (MOG)-IgG as biomarkers for two separate central nervous system inflammatory demyelinating diseases has revealed that neuromyelitis optica spectrum disorder (NMSOD) and MOG-IgG-associated disease (MOGAD) are responsible for clinically distinct subsets of ON. NMOSD-ON and MOGAD-ON both demonstrate tendencies for bilateral optic nerve involvement and often exhibit a relapsing course with the potential for devastating long-term visual outcomes. Early and accurate diagnosis is therefore essential. This review will summarize the current understanding of the clinical spectra of NMOSD and MOGAD, the radiographic and serological findings which support their diagnoses, and the current evidence behind various acute and long-term therapeutic strategies for ON related to these conditions. A particular emphasis is placed on a number of recent multi-centre randomized placebo-controlled trials, which provide the first level I evidence for long-term treatment of NMOSD.