Acute necrotizing encephalopathy (ANE) is a rare form of acute encephalopathy, predominantly occurring in childhood, which has a typical radiological phenotype including bilateral, symmetrical, diffusion-restricted lesions of the thalami; posterior putamen; cerebellum; and brainstem. To date, no study has systematically examined the long-term cognitive and psychological impact of ANE. The current study describes the neuropsychological outcomes of three paediatric cases of ANE, ranging from 18 months to 10 years post ANE. All three cases displayed inattention, fine motor difficulties and anxiety. Social difficulties were also reported in all cases. The severity of long-term impairment was associated with acute presentation, as well as convalescent neuroimaging. These findings highlight the need for detailed neuropsychological assessment and long-term rehabilitation.