Hereditary hemorrhagic telangiectasia (HHT), also called Rendu-Osler-Weber syndrome, is a rare autosomal dominant multisystemic vascular disorder, characterized by widespread mucocutaneous teleangiectasias, frequent visceral arteriovenous malformations (AVM) and a tendency for bleeding. This diagnosis should be suspected in all dermatological patients with generalized mucocutaneous vascular lesions at sites of predilection, associated frequent epistaxis and a positive family history. The aim of this paper is to emphasize the importance of a multidisciplinary approach, the role and timely cooperation of dermatologists and otorhinolaryngologists in the early clinical recognition and diagnosis of the disease. We present a family case of a 63-year - old patient with typical clinical features of HHT and long-standing multisystemic complications of unrecognized disease.