CCM research currently focuses on three main disease mechanisms: (1) clonal expansion of endothelial cells with biallelic inactivation of CCM1, CCM2, or CCM3, (2) recruitment of cells with preserved CCM protein expression into the growing lesion, and (3) disruption of endothelial cell–cell junctions in CCMs. Here, CCM2 is linked to cerebrocostomandibular syndrome.