Many recent reports of acute cerebellitis with a critical or potentially critical course have changed the conception of the disease from an acute cerebellar ataxia with a benign course to a notable disease entity with a heterogeneous pathogenesis. The most characteristic finding by magnetic resonance imaging is diffuse cortical swelling of the cerebellum, often complicated by hydrocephalus or tonsillar herniation. A timely assessment and appropriate choice of treatment, including surgical intervention when indicated, are key to improving the outcome of affected patients.