Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia is an arteriovenous malformation of different organs and systems, due to fibrovascular displasia. The knowledge of its molecular genetic basis has developed in the last few years. Generally, these patients are evaluated each time they have hemorrhagic manifestations as isolated events, rather than integrating these events into a single disorder. This is the case of a patient who had several skin and visceral manifestations years before the diagnosis was made.