Epithelioid hemangioendothelioma (EHE) is one of the rarest vascular tumor found in the brain. In the pediatric group of patients, the proportion of EHE is only 0.02% of all brain neoplasms, and therefore oncogenesis and the causes are currently not well understood. Intracranial localization of EH E is extremely rare, nevertheless, this type of neoplasm should be included in the differential diagnostic series when multiple small intracerebral masses with hypointense M R signal in T2 mode are detected on magnetic resonance imaging. No specific therapy is currently developed for this disease, the optimal treatment is surgical removal of the volumetric mass if this type of treatment is possible. This article presents a clinical case o f a primary multiple CNS lesion in a 14-year-old child with a long history of epilepsy. According to our data, the presented clinical case is the only one registered in the world, with primary multiple CNS EH E of the brain in the pediatric population. © 2022, Graphica Ltd. All rights reserved.