Von Willebrand disease (VWD) is the most common hereditary blood-clotting disorder in humans. It stems from a quantitative or qualitative deficiency of the von Willebrand factor (VWF). Von Willebrand factor is a blood multimeric protein whose function is closely related to blood-clotting, primarily platelet adhesion and factor VIII stabilization. VWD has the highest prevalence of all blood-clotting disorders, being reported as high as 1 in 100 individuals, though most of them do not have any symptoms. Clinically significant cases have a smaller prevalence, roughly one per 10,000. Hereditary VWD can be divided into 3 types: Type 1 VWD (quantitative disorder), type 2 VWD (many subtypes, qualitative disorder) and type 3 VWD (complete lack of blood VWF). Diagnostics of VWD is complex and requires several specific laboratory procedures like quantitative and qualitative VWF tests, as well as determining factor VIII levels. Though genetic testing can be done to diagnose VWD it is generally used only in specific cases, for example in order to differentiate between type 2N VWD and hemophilia A. In patients with type 1 and type 2A VWD, desmopressin, a synthetic vasopressin analogue, is used to boost VWF levels before dental or surgical procedures or in cases of minor trauma. Desmopressin in these patients causes already synthesized VWF to be released from endothelial cells. Antifibrinolytic therapy such as tranexamic acid is also used in such patients, alone or together with desmopressin. For patients with other types of VWD or patients with severe clinical symptoms, concentrates of VWF and FVIII are used. Prophylaxis can also be conducted using these concentrates in patients where we can expect significant hemorrhages. In women with heavy menstrual bleeding, estrogen and progesterone hormone therapy is used to reduce the frequency and duration of the menstrual periods. In some patients without previous bleeding diathesis acquired von Willebrand syndrome can occur.