Approximately 10% of newborns exhibit infantile hemangiomas, which are the most prevalent benign tumor. Pascual-Castroviejo was the first to document the correlation between facial hemangiomas and related cerebrovascular anomalies in infants. Building upon this, Frieden and colleagues introduced the term “PHACE association” in 1996 to characterize these observed patterns linked to substantial facial hemangiomas. The acronym PHACE includes the following features: posterior fossa malformations, facial hemangiomas, arterial cerebrovascular anomalies, cardiovascular anomalies, and eye anomalies. Recently, the acronym has been expanded to encompass ventral developmental anomalies, including sternal defects or supraumbilical raphe, leading to the designation PHACES association (Fig. 1) (Kim et al. 2012). PHACE syndrome manifests in approximately 2–3% of cases where infantile hemangioma is present. Documenting more than 300 cases of PHACE syndrome establishes it as among the most prevalent neurocutaneous vascular conditions in childhood. Several research endeavors indicate that when facial infantile hemangioma presents, there exists a notable 20–31% probability of it co-occurring with PHACE syndrome. Notably, PHACE syndrome displays a higher prevalence in females, with a ratio of nine to one compared to males (Rotter et al. 2018).