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PHACE syndrome refers to the association of large segmental facial hemangiomas with 1 or more of the following anomalies: posterior fossa malformations, arterial anomalies, cardiac anomalies, and eye abnormalities. In this review, we present a newborn with a large segmental facial hemangioma and abnormal genesis of the cerebropetal arteries. Furthermore, we give an overview of the anomalies associated with the PHACE syndrome. Patients with large segmental facial hemangiomas are at risk for 1 of these anomalies and should be investigated accordingly. We present a clinical algorithm for screening of patients with large segmental hemangiomas suggestive of the PHACE syndrome.