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Acute cerebellar ataxia is the most common cause of acute ataxia in children and it usually runs a self-limiting and ultimately benign clinical course. A small proportion of children have evidence of inflammatory swelling in the cerebellum. Many of these children suffer more severe and potentially life-threatening forms of cerebellar ataxia and may need more intensive treatments including urgent neurosurgical treatments. This more severe form of acute cerebellar ataxia is often termed acute cerebellitis. Many children with acute cerebellitis have long-term neurological sequela and evidence of structural cerebellar changes on follow-up imaging. Several patterns of cerebellar inflammation have been described. The authors describe the variabilities in the clinical and radiological patterns of disease in the cases that have been described in the literature.