The von Willebrand's Disease affects about 1-3% of the population and it is undiagnosed in most people. Originally described by the Finish physician Erik von Willebrand in 1926 this disorder is associated with mucous membrane bleeding including epistaxis, hypermenorhoea and excessive bleeding from surgery and dental procedures. In von Willebrands disease the patients have a quantitative or qualitative abnormality in the von Willebrand Factor (vWF) which has two major functions: it serves by bridging between platelets and injury site in the blood vessel wall, and it circulates as a complex with factor VIII, protecting it from rapid degradation. More than 20 different types and subtypes of vWD have been described but almost all patients can be classified as having 1 of the 2 most common types of vWD. About 70% of patients have type 1 and about 10-20% present themself with type 2A and 2B vWD type 3 in about 10%. By taking a clinical history of bleeding it is important to think about vWD and to make the appropriate diagnosis. If the vWD is suspected diagnostic tests should include an activated partial thromboplastin time, bleeding time, factor VIII:C, Ristocetin cofactor, and vWF antigen. Additional testing of ristocetin induced plattlet adhesion (RIPA), the multimeric structure and collagen binding test and genanalysis allow diagnosing the different types of v. Willebrand Disease. The treatment of choice in mild forms is the synthetic agent desmopressin. In patients with severe type 1, type 2B, 2N and type 3 or in people who do not response to desmopressin, the appropriate treatment is a factor VIII concentrate that is rich of vWF. Until now no recommendations in the orthopaedic literature have been found regarding diagnosis and treatment of vWS patients. Based on the experience with 43 orthopaedic patients presenting vWD over the last 15 years a contemporary guideline for the successful perioperative management of vWD in orthopaedic surgery is presented. In a close collaboration between the orthopaedic surgeon and the specialized haemostaseologist every invasive procedure in patients with vWD can be undertaken without the risk of excessive bleeding.