von Willebrand's disease (VWD) is the most common hereditary bleeding disorder. Unchecked or improperly managed, VWD-associated hemorrhage can lead to catastrophic surgical outcome. Based on the authors' recent experience with 21 procedures in 12 patients, a contemporary protocol for successful perioperative management of VWD in otolaryngologic surgery is presented. In patients with VWD type 1 or 2a, desmopressin, a synthetic vasopressin analog, is administered both pre- and postoperatively to release von Willebrand factor (VWF) from storage sites. In type 2b or 3, a factor VIII concentrate rich in VWF is administered. In addition, a 10- to 14-day course of intravenous and/or oral Amicar (Immunex Corp., Seattle, WA) may be prescribed postoperatively. Intraoperatively, the surgical laser is used to further decrease blood loss and augment hemostasis. This medical and surgical protocol minimizes the risk of hemorrhage and of transfusion-related complications through the judicious use of preoperative and postoperative coagulation replacement products. Using these guidelines in a variety of otolaryngologic cases, the authors have had no bleeding complications at their institution.