The Rendu-Osler-Weber disease is an hereditary hemorrhagic dysfunction with muco-cutaneous telangiectasia of diverse localization, being able to internal organs and committing the life in some cases, not alone for the derived systemic complications, but for the quantity of the recurrent nosebleed that are the most frequent feature and sometimes restrictive that these patients present. We report two family groups with multidisciplinary diagnosis, and analyze them most important ENT aspects for this syndrome.

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