Genetic variants in the EPHB4 gene are estimated to be present in approximately 10% of arteriovenous cerebral shunts, and the percentage of genetic-related cases of VGAM/VGD in the context of capillary malformation–arteriovenous malformation (CMAVM) or hereditary hemorrhagic telangiectasia (HHT) is considered to be even higher (Tas et al., 2022; Bayrak-Toydemir et al., 2011). Here, EPHB4 is linked to capillary malformation.