The antiphospholipid syndrome (APS) is an acquired autoimmune disease characterized by the association of thrombosis and/or pregnancy morbidity in patients with a persistently increased titre of antiphospholipid antibodies (aPL). Several laboratory tests recommended in the latest criteria, including phospholipid-dependent coagulation tests for detection of the lupus anticoagulant and ELISA for measuring anticardiolipin antibodies (aCL), still show methodological and diagnostic shortcomings. Despite advances during the last twenty years, a final consensus in therapy has not been reached. The article presents current recommendations for diagnosis and treatment of APS based on the most recent reports from the field of immunodiagnostics and the best documented recommendations in primary and secondary prevention of thrombotic complications and pregnancy morbidity (Tables I-IV). This paper also presents guidelines for particular situations, for example perioperative care for patients undergoing surgery or invasive procedures (Tables V, VI).