Hereditary hemorrhagic telangiectasia (HHT) is described as a multisystem vascular dysplasia characterized by multiple arteriovenous malformations (AVMs). It is an autosomal-dominant disorder caused by a mutation of at least 1 or 2 genes. AVMs that are void of intervening capillaries result in direct connections between arteries and veins. Small AVMs, or telangiectases that appear close to the surface of the skin or mucous membranes often rupture and bleed after slight trauma to the affected area. AVMs can be superficial to the more severe cases of GI bleeding, cerebral hemorrhage, and major pulmonary shunting. Shunting of air, thrombi, and bacteria through pulmonary AVMs may cause transient ischemic attack, embolic stroke, or cerebral abscess. HHT occurs in all ethnic and geographic areas, and the overall incidence of HHT in North America is estimated to be about one in five thousand people. The University of Pennsylvania Medical is one of eight centers in North America that has been designated as an HHT Center for Excellence by the HHT Foundation International. The Division of Medical Genetics at Penn works closely with diagnostic radiology, CT and MRI scanning, neuroradiology, neurology, pulmonary medicine, and, when appropriate, coordinates embolization procedures with the interventional radiology department. Patients with HHT receive comprehensive care for the diagnosis, management, and treatment of their disease at the University of Pennsylvania Medical Center. This presentation included information on the disease physiology as well as modes of definitive diagnosis via clinical presentation, diagnostic radiology, and genetic testing. The University of Pennsylvania Medical is one of two centers in the United States that performs genetic testing for mutations in either the endoglin or ALK 1 genes. Case study presentations include angiography films of pulmonary embolizations and patient interviews and specifically center around nursing implications and special care that these patients need from all healthcare professionals.