Link of Video Abstract: https://youtu.be/b4jB7Jos8tw Background: Ewing’s sarcoma is the second most common primary bone tumor presenting in childhood and adolescence. Ewing's sarcoma of the skull bones is an unusual occurrence (6 to 9% of all Ewing’s. sarcoma cases). Here, we present a rare case of Ewing's sarcoma involving the central nervous system (CNS) in a 13-month-old child and share our experience with its management. Case Presentation: We report a case of a 13-month-old girl with a chief complaint of gradually progressive head size. There is a history of seizures once a week before admission. The head CT scan with contrast administration revealed non-communicating hydrocephalus with bone defect in the occipital region and an intraaxial tumor of the posterior fossa due to Ewing’s sarcoma. Conclusion: Primary cranial Ewing's sarcoma of the cranium is a rare occurrence; its extension to intraparenchymal is even rare. This diagnosis can be considered in the differential diagnosis in children with a tumor involving the skull with destruction of the bone.