VWD is the most common inherited bleeding disorder, although most patients are asymptomatic. A personal and family history of bleeding (particularly recurrent epistaxis and heavy menstrual bleeding) may be the only key to preoperative diagnosis and prevention of excessive perioperative bleeding. Perioperative management of patients with VWD requires a multidisciplinary team approach. Pharmacologic prophylaxis for VWD consists of increasing endogenous levels of vWF (von Willebrand factor) with desmopressin (DDAVP), administering recombinant factor von Willebrand (vWF) concentrate, or administering plasma-derived factor VIII-von Willebrand factor concentrate (fVIII-vWF), along with adjuncts including topical agents. Patients with stable type 1 VWD undergoing a minor procedure are sometimes managed with DDAVP alone, although DDAVP is contraindicated in children <2 years. Acute bleeding episodes treated with coagulation factors may be monitored using viscoelastic testing of whole blood or conventional activated partial thromboplastin time (aPTT) because factor-level results may be late or unavailable. If specialized testing and medications are required, procedures in patients with VWD should be performed within reach of a Hemophilia Treatment Center (HTC).