The antiphospholipid syndrome (APS) is a complex autoimmune -disease characterized by the expression of antiphospholipid -antibodies (APL) and a variety of clinical presentation. The latest classification defines APS by the occurrence of vascular thrombosis and/or typical obstetrical morbidity together with persistently -detectable APL at least 12 weeks apart. The latest recommendation proposes a risk profile based on the type and titer of APL detected, in order to guide the intensity of prophylactic measures. Based on current knowledge, novel oral anticoagulants should not be used in APS, particularly in patients with a high-risk APL profile or arterial thrombosis. Beyond the mere aspect of anticoagulant treatment, immunomodulatory approaches to the APS such as hydroxychloroquine are under investigation.