GNAQ, encoding the G protein subunit alpha q, is somatically mutated in isolated capillary malformation termed as port-wine stain (PWS) and the more severe neurocutaneous Sturge–Weber syndrome (SWS) that is characterized by facial port-wine stains, and ocular and cerebral vascular malformations [16, 17]. Here, GNAQ is linked to Sturge-Weber syndrome.