Osler-Rendu-Weber (hereditary hemorrhagic telangiectasia) disease is an uncommon disease characterized by the presence of abnormal telangiectasias and arteriovenous malformations that cause recurrent episodes of bleeding. We present a patient with Osler-Rendu-Weber disease, with a history of multiple major bleeding events and severe aortic valve stenosis, who underwent aortic valve replacement. Unexpectedly, the postoperative course was uneventful, and there was no untoward bleeding in the early or in the late postoperative follow-up.