Hemorrhagic Hereditary Telangiectasia (HHT) disease, also called Osler-Weber-Rendu (OWR) disease, is a rare and underdiag-nosed genetic disorder characterized by a multisystemic vascular dysplasia. Nosebleeds, acute or chronic digestive tract bleeding and various problems due to the involvement of major organs (liver, lungs, brain) characterize the disease.Although it was described at the beginning of the 20th century, many patients, GPs and specialists still ignore the disease, its morbidities and the modalities of the treatment.That is the reason why the authors have decided to publish this review on this familiar, evolving and potentially life-threatening disease, whose management can be sometimes a real nightmare for the clinician.