Von Willebrand disease (vWD) is a common bleeding disorder caused by quantitative or qualitative defects of the von Willebrand factor (vWF). The clinical management of vWD has evolved over the past few decades, but it continues to pose a diagnostic and therapeutic challenge. In current practice, desmopressin is the treatment of choice for type 1 vWD because it corrects the FVIII/vWF levels. However, in patients with type 3 vWD and some forms of type 1 and 2 vWD, desmopressin is not effective, and for these patients, replacement therapy containing FVIII and vWF concentrates are the mainstay of treatment.