Hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) is a systemic fibrovascular dysplasia autossomal dominant, recognized as a classic triad of telangiectasia in face, hands and oral cavity, recurrent epistaxis and family occurrence. In this study, we report two patients with Rendu-Osler-Weber disease with recurrent epistaxis and cutaneomucous telangiectasias. Clinical manifestations, diagnostic and therapeutic features in the hereditary hemorrhagic telangiectasia are reviewed.