Background: PHACE syndrome (Posterior fossa malformations-hemangiomas-arterial anomalies-cardiac defects-eye abnormalities-sternal cleft and supraumbilical raphe syndrome) is a rare and serious neurocutaneous disorder. Cerebrovascular and cardiac abnormalities are the most important anesthetic concerns for patients with PHACE syndrome in order to provide safe and sufficient perioperative anesthesia care. Case Presentation: The present case discussed anesthetic implications of an infant boy who had PHACE syndrome and presented for elective laparoscopic-assisted pull through procedure under general anesthesia. Conclusion: Certain perioperative anesthetic implications should be considered mainly for difficult airway management that related to airway hemangiomas, abnormal cerebrovascular structure mainly carotid arteries involvement that affect CBF and results in cerebral ischemia and the association of congenital cardiac disease.

Keywords: PHACE syndrome, anesthetic considerations, case report