Cognitive dysfunction has been demonstrated in multiple sclerosis but has not been extensively studied after acute disseminated encephalomyelitis (ADEM). Because ADEM often presents with widespread demyelination, which may not completely resolve, these patients may be at risk for persistent cognitive dysfunction. The study objective was to explore the profile and severity of neurocognitive sequelae in pediatric ADEM. Children aged 6-15 years diagnosed with ADEM were invited to participate in a structured neurologic assessment, neuropsychological evaluation, and a follow-up magnetic resonance imaging. Nine of 15 children diagnosed with ADEM met the age criteria and six participated in the study. The mean age at presentation was 7.7 years; the mean duration of follow-up was 3.5 years. As a group, these children with prior ADEM performed within the average range on cognitive testing. However, a variety of mild cognitive deficits were demonstrated in each of the children, even in those whose magnetic resonance imaging studies had completely normalized. Four children demonstrated a cognitive profile of relatively poorer visuospatial/visuomotor function. The cognitive deficits observed in these children are similar but less severe than those previously reported in adults and children with multiple sclerosis, which may reflect the monophasic nature of ADEM, compared with the chronic, recurrent demyelination characteristic of multiple sclerosis.