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Vertex cephaloceles (VCs), also known as midline parietal cephaloceles, are among the most common midline scalp masses. Usually composed by a meningeal wall herniating from a vertex skull defect and covered by skin, VCs may also contain both anomalous vessels and neural elements. In spite of their harmless appearance, VCs often hide complex intracranial venous and/or brain malformations so that they represent a "tip of the iceberg". Vertical embryonic positioned straight sinus, elongation of the vein of Galen, persistence of the falcine sinus, fenestration of the superior sagittal sinus, corpus callosum agenesis, intracranial cysts, tentorial malformations, cerebellar vermis agenesis, hydrocephalus, and gray matter heterotopia are some of such associated anomalies.The treatment of VCs is surgical. It is indicated to prevent the rupture of the malformation or in case of pain or cosmetic impact. A careful preoperative radiological work up is mandatory to investigate the relationship between the VC and the sagittal sinus and/or the possible communication with the brain. The surgical procedure is usually carried out without significant complications.The prognosis of VCs is good even though the overall outcome is affected by the associated brain malformations.