Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is a malignant small, round cell tumor arising from bone and soft tissue in children and young adults. It can occur at osseous and extraosseous sites. Its usual locations are diaphysis of long bones followed by pelvis, ribs, vertebrae, and rarely skull. We reviewed the literature and PubMed advanced search on ES/pPNET occurring at extraosseous sites, mainly involving the central nervous system (CNS). We reported a case of a 22-year-old male presenting with seizure finally diagnosed as a case of ES/pPNET. The challenges in management of this rare CNS tumor and its differential diagnosis are highlighted. We found that most cases of ES involving CNS represent secondary metastases from extracranial sites of ES/pPNET and there are rare case reports of primary intracranial ES-pPNET. Furthermore, among these intracranial tumors, most common tumors occupy an intraaxial location and only a handful of cases of dural-based or extraaxial tumors mimicking meningioma are reported. Differentiation of pPNET from central PNET (cPNET) is important as it has definitive therapeutic and prognostic implications. Awareness of this entity of ES/pPNET, its rare dural presentation, and differentiation from the more common cPNET is needed for appropriate patient management. Meningeal ES/pPNET has to be kept in mind in the differential diagnosis of meningeal tumors eroding bone.

Encephalocele is a rare lesion, being an embryological mesodermal anomaly which results in a defect in the cranium and dura, associated with herniation of meninges, cerebrospinal fluid, or brain tissues through a defect usually covered by scalp. Surgical management of children with giant occipital encephalocele requires careful attention to pediatric anesthetic and surgical principles. We present a case of a giant occipital encephalocele highlighting the problems encountered in its management.

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