Purpose or Case Report: PHACE syndrome (P: posterior fossa malformations, H: haemangioma, A: arterial anomalies, C: coarctation of the aorta and cardiac defects, E: eye abnormalities, S: sternal defects) is one of the neurocutaneous disorders. It describes a constellation of abnormalities that can occur in association with segmental craniofacial infantile haemangioma, which is the hallmark of this syndrome. Intracranial anomalies are the most common extracutaneous feature of PHACE syndrome. Typical intracranial malformations include posterior fossa malformations like Dandy-Walker complex and cerebellar hemispheric hypoplasia ipsilateral to the hemangioma, arterial anomalies like agenesis, aneurysms, arterial kinking or coiling, Moyamoya-like collateralization and persistent embryonic arteries. Less frequent findings include cortical dysplasia and cortical migration anomalies, dysplasia of the corpus callosum, intracranial capillary hemangiomas and arachnoid cysts.

We present 3 cases which we think significantly contribute to the growing recognition of relatively unique intracranial associations that are likely important for the diagnosis of PHACE syndrome.

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