Hemophilia A and Villebrand's disease owe similar clinical features, for their treatment the preparations, containing factor VIII and Villebrand's factor are used. Preoperatively it is necessary, first of all, to establish the disease diagnosis and its form definitely and then an optimal scheme of hemostatic therapy must be selected with its intraoperative and postoperative correction under control of laboratory monitoring. It is important to reveal timely a hemostasis disorders and a hemophilia of inhibitory form occurrence. Modern rational methods of prophylaxis and treatment of hemorrhage, using desmopresin (for mild forms of hemophilia A and Villebrand's disease type III) as well as preparations of restoration therapy, containing factor VIII and Villebrand's factor, are recommended. Preparations of auxiliary therapy are applied according to certain indications present for elimination of vascular-thrombocytic disorders, fibrinolysis activation, hemophilia of inhibitory form and hormonal disorders occurrence. It is also stressed, that, while these diseases are present, it is necessary to follow direct indications for the operations performance and guaranteeing a reserve of sufficient quantity of corresponding hemostatic preparations.