PHACE is a newly named neurocutaneous syndrome involving the skin, brain, eyes and ventral side of the body and is the most common complication of facial segmental IH. PHACE is a combination of acronyms and includes posterior cranial fossa vascular malformations, facial angiomas, arterial anomalies, aortic stenosis and/or cardiac defects, ocular anomalies and sternal fissures. According to the diagnostic criteria published in 2009, patients are classified into 3 risk levels based on the organ(s) involved at the time of presentation: definite, likely, and probable. close to 90% of patients with PHACE are women. However, unlike non-syndromic IH, patients with PHACE are mostly normal for gestational age and weight at birth and also tend to have singleton pregnancies. A prospective study of 108 patients with facial segmental IH found that nearly 1 in 3 met the diagnostic criteria for PHACE. All patients with segmental facial IH should be evaluated with magnetic resonance imaging (MRI) or magnetic resonance angiography (MRA) at or before the visit, as structural brain and cerebrovascular abnormalities are the most common extracutaneous manifestation of abnormalities in patients with PHACE, with an incidence of 72% to 94%. Lateral frontal (zone 1) or mandibular (zone 3) areas of IH were most closely associated with brain involvement. The incidence of cerebral vascular anomalies was in the order of dysplasia (56%), angiogenesis and developmental anomalies (47%), vascular stenosis (39%), failure to visualize due to vascular occlusion or dysplasia (20%), and undegenerated embryonic arteries (20%). As in Sturge-Webe syndrome, the vast majority of cerebrovascular malformations occur ipsilateral to the cutaneous IH. Of these, the internal cerebral artery is the most commonly involved artery. Structural brain anomalies are less common than vascular anomalies, with posterior cranial fossa malformations (developmental anomalies) being the most common. Permanent neurological impairment includes developmental delay, seizures, headaches and strokes. Of the cardiovascular anomalies that accompany PHACE patients, 41% present as aortic arch, intracardiac, or cephalic-arm vascular anomalies, with abnormal pulsation of the subclavian artery (21%) being slightly more common than constriction (19%). Ninety-two percent of patients with cardiac involvement have cerebrovascular or cervical vascular abnormalities, a known risk factor that increases the likelihood of cerebrovascular accidents. the vascular narrowing seen in PHACE syndrome is significantly different from normal vascular narrowing, characterized by large transverse arch narrowing and adjacent aneurysmal dilatation sites. 7% to 17% of patients with PHACE have ocular abnormalities, including abnormalities of the optic disc in petunia, Posterior segment anomalies such as optic nerve hypoplasia, retinal vascular anomalies, embryonic vascular nondegeneration, defects, and pars plana staphyloma. Anterior segment abnormalities such as cataracts and microphthalmia are secondary criteria for diagnosis, and others have suggested that congenital glaucoma be included in the diagnostic criteria. Supraumbilical cleft and sternal cleft are associated in more than 15% of patients, while arteriovenous malformations are rare. Lower body disorders associated with PHACE syndrome include lipomas, genitourinary anomalies, ulcers, myelopathy (spinal cord tethering is the most common extracutaneous manifestation), bone malformations, anorectal malformations (anal atresia is the most common), arterial anomalies, and renal abnormalities. ultrasound screening of the abdomen, pelvis, and spine is necessary in children younger than 3 months of age, while children older than 3 months of age should be MRI and MRA based on skin IH and other abnormalities. Patients with PHACE can be treated with oral propranolol at a dose of 1.8 mg/kg , administered orally in 2 to 3 doses for a mean duration of 12.3 months. To prevent induction of stroke, the dose of the drug is administered as low as possible, slowly increasing the dose in 3 oral doses to prevent sudden changes in blood pressure, with close follow-up and evaluation, including neurology consultation.