von Willebrand disease (VWD) is the most common inherited bleeding disorder, caused by a quantitative or qualitative defect of von Willebrand factor (VWF). This factor is essential for platelet-dependent primary hemostasis and also plays a role in secondary hemostasis by carrying and protecting FVIII. Affected persons may experience varying degrees of mucocutaneous bleeding or heavy menstrual bleeding, which can be the first and only sign of the disease. VWF levels can fluctuate due to stress from acute bleeding, anemia, pregnancy, or medication. The diagnosis of VWD is confirmed when VWF levels are below 30 IU/dL or between 30 and 50 IU/dL with a positive bleeding history. Genetic testing is important in defining the VWD subtype.