Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant demyelinating disorder. It is considered to be the most severe form of acute disseminated encephalomyelitis. It is associated with a high rate of mortality or severe neurological deficits, with only few reports of recovering patients. We report a 49-year-old female patient who presented with acute onset of neurological deficits, due to a right thalamic hemorrhage together with intraventricular bleeding. There was a continued clinical deterioration. In follow-up CT and MRI scans bilateral confluent white matter lesions were seen, together with T2 * hypointense lesions indicating microbleeds. A stereotactic biopsy was carried out and the diagnosis of AHLE was suggested. In acute post infectious encephalitides (and especially the severe form) early diagnosis, administration of treatment is of prime importance for outcome in a patient with Hurst encephalitis.