Background: Myelin oligodendrocyte glycoprotein antibody-associated disease myelin oligodendrocyte glycoprotein antibody-associated disease is an emerging demyelinating condition distinct from neuromyelitis optica spectrum disorder and multiple sclerosis affecting both children and adults with a spectrum of clinical manifestations ranging from optic neuritis, myelitis and acute disseminated encephalomyelitis.Case report: We describe the case of a 2 year-old girl diagnosed with myelin oligodendrocyte glycoprotein antibody-associated disease presenting as recurrent acute disseminated encephalomyelitis who showed suppression of disease activity after treatment with rituximab. This case represents the youngest Mexican patient with myelin oligodendrocyte glycoprotein antibody-associated disease reported to date.Conclusions: Information regarding the clinical presentation and evolution among different population could help to a better understanding of the presentation of this entity.