The patient was a 6-year-old girl with clinically isolated syndrome-like anti-myelin oligodendrocyte glycoprotein-associated disease (MOG-AD). Methylprednisolone pulse therapy resolved her cerebral lesion, and her visual acuity and field fully recovered after plasma exchange. This is the first case report presenting the therapeutic course in a child with clinically isolated syndrome-like MOG-AD. [J Pediatr Ophthalmol Strabismus. 2023;60(2):e11-e15.].