BACKGROUND: We present two cases of children who were diagnosed with cerebellitis with acute cerebellar swelling. This rare pathology is potentially fatal, and no clear treatment guidelines are described in the literature. DISCUSSION: Considering our experience, we discuss the different therapeutic strategies and propose aggressive surgical measures consisting of external ventricular drainage and posterior fossa decompression in case of failure of early response to medical treatment to limit secondary cerebellar and brainstem lesions.