MOG antibody-associated disease (MOGAD) is a demyelinating disorder distinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). MOGAD most commonly presents with optic neuritis (MOG-ON), characterized by bilateral involvement, optic disc swelling, and longitudinally extensive optic nerve hyperintensity on MRI. Serum MOG IgG detected via live cell-based assays is highly specific for MOGAD. Key clinical and radiological features aid diagnosis, while ancillary tests like visual fields and CSF analysis are less discriminatory. Optical coherence tomography (OCT) identifies optic nerve swelling acutely and atrophy chronically, serving as a potential biomarker. MOG-ON responds well to corticosteroids, but relapses are common, especially with early steroid tapering. Optimal acute therapy and long-term maintenance immunosuppression are critical for relapse prevention. MOGAD has a biphasic age distribution, with peak onset in children 5–10 years and adults 20–45 years. MOG-ON is the most common initial manifestation in adults, followed by transverse myelitis. MOGAD is associated with various ophthalmic presentations, including uveitis, peripheral ulcerative keratitis, and optic perineuritis. MRI is the preferred imaging modality, with key features like longitudinal optic nerve involvement and retrobulbar enhancement distinguishing MOG-ON from other demyelinating disorders. Visual evoked potentials (VEPs) show delayed P100 latencies and reduced amplitudes in MOG-ON. CSF findings are non-specific but may include pleocytosis and oligoclonal bands. MOG IgG testing is crucial for diagnosis, with live cell-based assays being more sensitive and specific. MOGAD has a favorable disease course with relapse rates up to 70% over five years, but long-term steroid use is associated with risks. Maintenance immunosuppression, such as intravenous immunoglobulin or rituximab, is being explored for relapse prevention. MOGAD is distinct from other autoimmune disorders, with unique clinical and pathological features. Treatment strategies must balance acute management with long-term relapse prevention, considering individual patient factors.MOG antibody-associated disease (MOGAD) is a demyelinating disorder distinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). MOGAD most commonly presents with optic neuritis (MOG-ON), characterized by bilateral involvement, optic disc swelling, and longitudinally extensive optic nerve hyperintensity on MRI. Serum MOG IgG detected via live cell-based assays is highly specific for MOGAD. Key clinical and radiological features aid diagnosis, while ancillary tests like visual fields and CSF analysis are less discriminatory. Optical coherence tomography (OCT) identifies optic nerve swelling acutely and atrophy chronically, serving as a potential biomarker. MOG-ON responds well to corticosteroids, but relapses are common, especially with early steroid tapering. Optimal acute therapy and long-term maintenance immunosuppression are critical for relapse prevention. MOGAD has a biphasic age distribution, with peak onset in children 5–10 years and adults 20–45 years. MOG-ON is the most common initial manifestation in adults, followed by transverse myelitis. MOGAD is associated with various ophthalmic presentations, including uveitis, peripheral ulcerative keratitis, and optic perineuritis. MRI is the preferred imaging modality, with key features like longitudinal optic nerve involvement and retrobulbar enhancement distinguishing MOG-ON from other demyelinating disorders. Visual evoked potentials (VEPs) show delayed P100 latencies and reduced amplitudes in MOG-ON. CSF findings are non-specific but may include pleocytosis and oligoclonal bands. MOG IgG testing is crucial for diagnosis, with live cell-based assays being more sensitive and specific. MOGAD has a favorable disease course with relapse rates up to 70% over five years, but long-term steroid use is associated with risks. Maintenance immunosuppression, such as intravenous immunoglobulin or rituximab, is being explored for relapse prevention. MOGAD is distinct from other autoimmune disorders, with unique clinical and pathological features. Treatment strategies must balance acute management with long-term relapse prevention, considering individual patient factors.