PHACE syndrome is emphasized through the characteristic findings of the posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and abnormalities of the eye. This distinctive syndrome requires a multidisciplinary care team to take care of individual problems. This review article focuses on the clinical presentation of PHACE syndrome: the initial approach from the classic cutaneous presentation and the extra-cutaneous anomalies found in association with the syndrome. The recent diagnostic criteria, management surveillance, treatment, and long-term follow-up are also addressed in this presenting review.