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We report the case of a middle childhood boy diagnosed with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), who was resistant to initial steroid pulse therapy and plasma exchange but achieved long-term remission following rituximab (RTX) treatment. Over a 4-year follow-up, the patient experienced no relapse, serum anti-MOG antibodies were undetectable and steroids were successfully tapered and discontinued. This case highlights the potential efficacy of RTX not only as a relapse-preventing agent but also as an acute-phase therapy in paediatric-onset MOGAD.