The purpose of this retrospective study is to document the natural history of epistaxis in patients with hereditary hemorrhagic telangiectasia. A telephone questionnaire was administered to 73 patients who had been previously screened for pulmonary arteriovenous malformations (PAVMs). The incidence of epistaxis in this population was 93%, with a mean onset age of epistaxis of 12 years, a mean frequency of bleeding of 18 episodes per month, and a mean duration of bleeding of 7.5 minutes. More than 90% of patients experienced the onset of epistaxis before the age of 21 and symptoms were progressive with age. There were no differences in the age of onset, frequency of epistaxis, or duration of epistaxis between patients with PAVMs versus those without PAVMs. Although the natural history of epistaxis does not predict the presence or absence of pulmonary arteriovenous malformations, epistaxis is an early marker of the disease, hereditary hemorrhagic telangiectasia, and might guide screening for pulmonary and cerebral arteriovenous malformations in children of affected parents.