In this chapter, the authors will outline the unique perioperative and anesthesia management strategies for pediatric patients with Apert syndrome. Preoperatively, it is imperative to optimize these medically complex patients before elective surgery. Early preoperative evaluation and timely medical management of anemia and comorbidities before elective procedures are crucial to ensure the best outcomes. Elective surgery should be postponed as other clinical imperatives permit until modifiable risks are appropriately addressed and optimized. General intraoperative anesthesia considerations in children with craniofacial syndromes such as Apert syndrome include difficult airway, difficult intravenous access, obstructive sleep apnea, bronchospasm, raised intracranial pressure, clinically significant blood loss, blood transfusion and its associated complications, as well as prolonged duration of surgery, intensive care stay, and hospital length of stay. Herein, specific perioperative anesthetic concerns and contemporary evidence-based management strategies will be outlined for each of the high-risk surgical procedures that the patient with Apert syndrome may present for, including craniofacial surgery: endoscopic strip craniectomy, open craniosynostosis surgery (fronto-orbital advancement and calvarial vault remodeling), and midface advancement surgery.