Since all variants show dominant inheritance with a 50% risk of transmission to the offspring and possible affection of multiple organs, genetic screening of EPHB4, RASA1, and the three HHT-associated genes has to be considered for a high-flow intracerebral vascular malformation, even if the patient does not have evocative skin abnormalities. The gene discussed is EPHB4; the disease is hereditary hemorrhagic telangiectasia.