The authors report the case of a 72-year-old woman with severe aortic stenosis who had a bleeding tendency develop due to type IIA acquired von Willebrand disease. She underwent aortic valve replacement with a 19-mm Freestyle stentless valve (Medtronic Inc, Minneapolis, MN). The postoperative course was uneventful and the bleeding tendency resolved. A review of this operative case from our institution demonstrated that aortic valve replacement was one of the most effective treatments of this disease, which can be potentially lifesaving.