Antiphospholipid syndrome (APS) is a systemic autoimmune disease which manifests as thrombotic and/or obstetric adverse events, mediated by persistent circulating antiphospholipid antibodies (aPL) detected by means of three tests: lupus anticoagulant, anticardiolipin and anti β2-glycoprotein I antibodies. It can be isolated or associated with other autoimmune rheumatic diseases. During pregnancy, patients with APS have a higher risk of obstetric complications and a higher thrombotic risk due to the pregnancy itself. Therefore, a preconception counselling is crucial to assist the patient and her family in planning the pregnancy and to optimise the management by implementing preventive measures that can allow the best outcomes for both the mother and the baby. In clinical practice, we can distinguish between different subsets of patients that require alternative approaches: patients with obstetric APS, patients with thrombotic APS, patients with APS associated with other autoimmune diseases, and asymptomatic individuals (aPL carriers). Pregnancy and foetal outcomes have greatly improved in the past 2 decades as a result of a therapeutic implementation based on individual risk stratification and a combination of low dose aspirin and heparin. Additional strategies have been suggested for women with pregnancy failure despite this management.