Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder characterized by laboratory abnormalities and clinical symptoms overlapping those observed in inherited von Willebrand disease. AVWS may occur associated with several conditions, mostly hematological disorders (lymphoproliferative disorders, monoclonal gammopathies, and myeloproliferative disorders) and cardiovascular diseases. A significant association with aortic valve stenosis and continuous-flow left ventricular assist devices (LVADs, up to 100%) is increasingly reported. The treatment is mainly based on the severity of presenting clinical phenotype and the underlying causative pathophysiology. Removal of the triggering condition via chemotherapy or surgery may completely resolve the disorder. Immunoglobulins may be indicated in patients with monoclonal gammopathies and recurrent bleeding, thus contrasting the accelerated removal of VWF-monoclonal IgG paraprotein complexes. Desmopressin and FVIII/VWF concentrates may be helpful to stop or prevent bleeding during invasive procedures, but the half-life of FVIII and VWF may be shortened, and thus accurate clinical and laboratory monitoring is required. Tranexamic acid is especially useful as co-treatment of mucosal bleeding.