This hypothesis was generated by the rare concurrent description of PPV with Sturge-Weber syndrome (SWS) (Chhajed et al., 2010; Vidaurri-de la Cruz et al., 2003), a vascular disorder with no pigmentary phenotype recently found to be the result of postzygotic mosaicism for activating mutations in GNAQ (Shirley et al., 2013). This evidence concerns the gene GNAQ and vascular disorder.