jats:pVon Willebrand disease is the commonest bleeding disorder encountered in the medical and surgical field. As a result of the repetitive bleeding into the joints many patients suffer from arthropathy that can cause a significant amount of pain and functional limitation. Total joint arthroplasty is being utilised as the last resort in managing these patients; however, the presence of von Willebrand disease raises a number of dilemmas. In this review, we look at the effects of von Willebrand disease on joints and the perioperative management of von Willebrand disease patients undergoing total joint arthroplasty.</jats:p>