Apert syndrome is a rare fibroblast growth factor receptor 2 (FGFR2) craniosynostosis syndrome associated with a high incidence of neurocognitive impairment. The neurocognitive deficits in Apert syndrome most likely relate to alterations in white matter structures of the brain, which in turn are linked to the expressivity of the FGFR2 gene mutation. Skull malformation, raised intracranial pressure (ICP), visual impairment, hearing deficit, and number of surgical procedures may exacerbate neurocognitive risk. Psychosocial and environmental factors such as family support, developmental and educational interventions, and surgical management for known medical risk factors, such as raised ICP, airway compromise, hearing loss, and exophthalmia, may moderate this risk. This chapter reviews the scientific literature on neurocognitive and adaptive behavioral outcomes in Apert syndrome, discusses the hypothesized etiology of neurocognitive impairment, and provides a template protocol for clinical management and empirically based research into Apert syndrome’s cognitive and behavioral characteristics.