The aim of this paper is to present a case of recurrent epistaxis diagnosed with hereditary hemorrhagic telangiectasia syndrome, also known as Osler-Weber-Rendu syndrome. With this case, we intend to show the relevance of including this syndrome in the differential diagnosis of epistaxis, a common symptom in our daily practice. CASE REPORT: R. R. S. R., a 48-year-old woman, was referred to the otolaryngology HUAP-UFF by the pulmonology department with a history of recurrent epistaxis and breathlessness. The patient had mild epistaxis in childhood with progressive worsening over the past 15 years. During this period, she often had to go to the emergency department for bleeding. She has 2 brothers and a grandfather with the same history. On examination, she presented with telangiectasias of the face, oral cavity, lips, and hands. Anterior rhinoscopy showed a point of recent bleeding that was cauterized. Complementary tests excluded the presence of visceral arteriovenous malformations. This syndrome is a disorder with autosomal dominant inheritance, characterized by multiple arteriovenous malformations that present clinically with recurrent epistaxis, telangiectasias, visceral lesions, and a family history of epistaxis in first degree relatives, requiring 3 of 4 characteristics to reach a diagnosis according to the criteria of Curacao. It is very important that the otolaryngologist, often the first professional to be sought, has both the ability to diagnose the syndrome and to handle it properly, as making an early diagnosis can reduce the damage caused by this disease.