Rendu-Osler-Weber disaese or hereditary hemorrhagic telangiectasia (HHT) is a multisystem autosomal dominant hereditary disorder. The disorder is manifested by multiple dysplasia of bloodvessels of the skin en mucous membranes. This results in recurrent and sometimes severe bleedings, of which epistaxis is the most common. Cardial, pulmonary and cerebral manifestations can be responsible for complications. A patient is presented with Rendu-Osler-Weber disease followed by a review of the literature.