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Acute cerebellitis is a rare inflammatory disorder that occurs most frequently in children. The typical clinical course of acute cerebellitis is benign. However, in some cases of acute cerebellitis, fulminant cerebellar swelling with obliteration of the fourth ventricle causes brain stem compression and acute obstructive hydrocephalus, which can be life-threatening and require emergent neurosurgical procedures. We describe the case of a 4-year-old girl whose acute cerebellitis caused brain stem compression, acute obstructive hydrocephalus, and death. The patient was admitted to the emergency department because of a severe headache that had persisted for 5 days. Neurological examinations revealed no specific abnormality. Brain magnetic resonance imaging (MRI) demonstrated diffuse swelling and high signal intensity lesions in the cerebellar hemispheres and vermis, obliteration of the fourth ventricle, and brain stem compression on T2-weighted images in conjunction with mild hydrocephalus. Cerebrospinal fluid (CSF) analysis revealed white blood cell and red blood cell counts of 180/mm3 and 0/mm3, respectively; protein and glucose concentrations of 263.6 mg/dL and 37 mg/dL, respectively; and negative culture results. Despite aggressive treatment, the patient developed sudden cardiorespiratory arrest on day 2. Although emergency neurosurgery was performed after cardiopulmonary resuscitation, her condition progressed to brain death, and she died on day 29. This case suggests that timely and appropriate neurosurgery should be actively considered to relieve increased intracranial pressure in the early phases of acute cerebellitis. [ABSTRACT FROM AUTHOR]