Among patients with autosomal dominant polycystic kidney disease (ADPKD), a disorder that affects the kidneys and other organs caused by mutations in PKD1 and PKD2, a wide spectrum of vascular abnormalities have been described including intracranial aneurysms (and dolichoectasias), thoracic aorta and cervicocephalic artery dissections, and coronary artery aneurysms [11,12]. Here, PKD1 is linked to autosomal dominant polycystic kidney disease.