Although many pathways and modifier genes are implicated in bAVM pathogenesis, in this review, we focused on recent discoveries in RAS-MAPK-ERK, TGFβ, platelet-derived growth factor b (Pdgfb) pathways, and non-cording RNAs in bAVM studies. This evidence concerns the gene TGFB1 and arteriovenous malformations of the brain.