The PHACES syndrome refers to coexistence of the Posterior fossa brain malformations, (Segmental Haemangiomas) of the face with (Arterial anomalie) and midline raphe defects (Sternal defects or Supraumbilical raphe). The diagnostic criteria have not been precisely established and are still discussed by the experts. The authors present current knowledge on PHACE syndrome and discuss suggested diagnostic criteria of PHACE syndrome.