Aortic valve stenosis can be complicated by recurrent gastrointestinal bleeding, particularly that due to angiodysplasia, also called Heyde syndrome. Recently, acquired type 2A von Willebrand disease, which is characterized by the loss of the large multimer of von Willebrand factor by the shear stress of aortic valve stenosis, was reported to be associated with this hemorrhagic syndrome. A 78-year-old woman, with severe aortic stenosis, presented with advanced anemia due to recurrent gastrointestinal bleeding and was diagnosed Heyde syndrome. By perioperative supplementation of von Willebrand factor and factor VIII, aortic valve replacement was safely performed without gastrointestinal bleeding. After the operation, the multimer of von Willebrand factor was normalized and thereafter no gastrointestinal bleeding occurred. This case reports the successful aortic valve replacement for Heyde syndrome, with confirmed hematologic recovery.