PHACE syndrome is a neurocutaneous disorder characterized by large cervicofacial infantile hemangiomas and associated anomalies of the brain, cerebrovasculature, aorta, heart, and eyes. Two categories of neurologic disease are observed among PHACE patients: congenital malformations of the cerebellum, cerebrum, and cerebral vasculature and progressive stenoses and occlusions of principal cerebral arteries. A subgroup of patients develops a moyamoya-like vasculopathy and consequent ischemic strokes. This report details the clinical course and management of a young female with PHACE and reviews the various neurologic aspects of this neurocutaneous disorder. This patient presented with high-grade stenoses of the internal carotid arteries bilaterally, formation of extensive vascular collaterals, and multiple ischemic strokes. She underwent bilateral pial-synangiosis procedures and has not had stroke recurrence in 2 years of follow-up. The presence of a characteristic infantile hemangioma necessitates further evaluation for the extracutaneous features of PHACE. In cases of steno-occlusive vasculopathy, we recommend early consideration of encephaloduroarteriosynangiosis or a commensurate revascularization procedure.