von Willebrand factor (VWF) is a complex plasma protein that promotes platelet adhesion to the subendothelial matrix of injured blood vessels and provides stability to factor VIII in the circulation. von Willebrand disease (VWD) is a haemorrhagic disorder of highly variable severity caused by qualitative and quantitative abnormalities of VWF. The approach to treating urgent bleeding in patients with VWD is dependent on an accurate diagnosis of the patient's type of VWD and knowledge of the expected responses to treatment modalities. These include administration of desmopressin (DDAVP), a vasopressin analogue that promotes the release of VWF and factor VIII (FVIII) from storage sites, and replacement therapy with a viral-inactivated concentrate containing normal VWF and FVIII. Monitoring of an individual's response in the non-bleeding state is required to determine the response to desmopressin that may be achieved and to determine the individual pharmacokinetics of infused VWF and FVIII. In this article, specific approaches to the choice of treatment modalities, calculation of dosages, monitoring of therapy, possible side effects and complications of the treatment of the various types of VWD will be discussed.