The antiphospholipid syndrome (APS) is characterised by a great clinical variability, with diverse clinical presentations such as venous thromboembolism, preeclampsia, arterial thrombosis and renal and cerebral small vessel thrombosis. Given this wide spectrum of manifestations, it is very important to make an early diagnosis in order to start adequate medical therapy before irreversible damage ensues. Persistent positivity of lupus anticoagulant (LA), anticardiolipin antibodies (aCL) at high levels or the combined triple positivity of LA, aCL and anti-ss(2)GPI make the diagnosis of APS likely in the adequate clinical setting. The treatment of APS is based on antiaggregant and anticoagulant drugs. The optimal approach is still debated; however, indefinite anticoagulation is warranted. We recommend high intensity anticoagulation in patients with arterial and/or recurrent events, as well as a strict control of vascular risk factors. Pregnant women with APS should be best attended in combined medical-obstetric clinics. Low dose aspirin should be given to every pregnant woman with antiphospholipid antibodies, with the addition of low molecular weight heparin in those with previous thrombosis, previous fetal death or failure of monotherapy with aspirin.