Due to an unusually wide frontal dehiscence in the neonatal period, the young infant with Apert syndrome rarely faces acutely raised intracranial pressure. In addition to the generally larger skull associated with bicoronal synostosis, there is often a ventriculomegaly which may be mistaken for a hydrocephalus, which is rarely present. Additionally, children with Apert syndrome frequently have associated brain malformations, including temporo-mesial anomalies, and hypoplasia or agenesis of the corpus callosum (CC). However, due to the absence of lambdoid synostosis, the herniation of the cerebellum tonsils (Chiari malformation) is rarely present. Prevention of turricephaly involves early surgical expansion, often with posterior expansion with distraction. Indications for needing a ventriculoperitoneal (VP) shunt are very rare. With clinically significant obstructive sleep apnea syndrome (OSAS), early frontofacial monobloc is preferred to a staged sequence involving fronto-orbital advancement which is reserved to the patients with moderate OSAS. Our cognitive data support the importance of a first surgical expansion before one year of age.