von Willebrand disease (vWD) is a bleeding disorder characterized by a complex hemostatic defect. Abnormal platelet function, usually reflected by a prolonged bleeding time, is the result of a quantitative or qualitative defect of von Willebrand factor (vWF). A secondary deficiency of factor VIII procoagulant protein (factor VIII) may occur leading to a coagulation defect as well. These two glycoprotein macromolecules circulate as a complex in plasma. This article will review current understanding of structures and functions of vWF factor and factor VIII as they relate to the pathogenesis, diagnosis, classification and therapy of vWD.