The acronym PHACES describes a rare neurocutaneous syndrome that comprises posterior fossa malformations, facial hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal defects. Facial hemangiomas constitute the hallmark of this disorder. Giant intracranial aneurysms have not been previously reported in the literature as manifestations of PHACES syndrome and can present difficult therapeutic challenges. The authors describe a unique case of a 13-year-old adolescent boy with an incomplete phenotypic expression of PHACES syndrome who harbored diffuse cerebral angiodysplasia and a giant fusiform internal carotid artery (ICA) aneurysm extending from the distal cavernous segment to the supraclinoid segment. The aneurysm was successfully treated with a high-flow saphenous vein graft bypass from the external carotid artery to the distal middle cerebral artery followed by proximal ICA occlusion. This case represents a unique vascular manifestation of PHACES syndrome that required a complex management strategy. The authors review the literature on this rare disorder and emphasize the importance of considering the diagnosis of PHACES syndrome in child with a facial hemangioma.