The antiphospholipid syndrome (APS) is characterized by a wide variability in clinical manifestations. Recommendations for therapy are conditioned by the lack of appropriate studies, due either to methodological limitations or excessive selection of patients. There is consensus in treating patients with APS and first venous thrombosis with warfarin to a target international normalized ratio (INR) of 2.3-3.0. However, a recent systematic review including observational studies found patients with APS and stroke to be at a high risk of recurrent events. We thus recommend a target INR>3.0 in this group. Likewise, the optimal approach for women with obstetric manifestations of APS is not completely defined; some authors recommend universal aspirin plus heparin whereas others consider aspirin in monotherapy useful for women with recurrent early miscarriage only. Correction of vascular risk factors and a high-risk management of pregnancy, including Doppler studies of the uterine and umbilical vessels, are warranted. Hydroxychloroquine and statins are likely to become important in the future.