摘要:Osler-Weber-Rendu syndrome (OWRS), also known as Hereditary Hemorrhagic Telangiectasia (HHT) is a rare, autosomal dominant, dysplastic vascular disorder that usually presents with subtle symptoms, causing frequent delays in diagnosis that lead to many complications, with arteriovenous malformations as the responsible for the highest morbidity and mortality. We present a case of a 51 year old female with history of multiple episodes of epistaxis that presents to the ED with severe headache and shortly thereafter, loss of consciousness. Workup included a CT scan of brain that demonstrated subarachnoid hemorrhage and an Angiogram, that depicted and anterior communicating artery aneurysm. Patient underwent endovascular treatment without complications and other findings included multiple nasal mucosal telangiectasias, left occipital dural arteriovenous Fistula and multiple pulmonary arteriovenous malformations. Diagnosis of HHT was confirmed by the Curazao criteria. Three month later after discharge from hospital she came back to the ED with severe hemoptysis and dyspnea. She died shortly that day without time for surgical treatment.