Antiphospholipid antibody syndrome (APS) is a state of hypercoagulability secondary to an autoimmune disorder. It is associated with thrombotic events in venous and arterial vessels, obstetric complications characterized by recurrent fetal losses, and increased perinatal morbidity. APS is classified as primary, when not associated with other pathologies; or secondary, when associated with an underlying autoimmune disease with, solid tumor, or hematological disorder. Clinical findings include livedo reticularis, thrombocytopenia or hemolytic anemia, maternal morbidity, and recurrent thrombotic episodes and others. Laboratory tests show circulating antiphospholipid antibodies (aPLs); however, even in the presence of these antibodies, patients can be asymptomatic. Estimates predict that about 5% of the populations have circulating aPLs, but the incidence of APS is only five cases per 100,000 people, as diagnosis of this syndrome requires clinical and laboratory findings to be simultaneously present. In cases of secondary APS, or in acute cases with imminent risk of death (as in catastrophic APS), it may be necessary to reduce aPL serum levels using immunomodulators, immunosuppressants, or plasmapheresis, in order to treat the associated pathologies. In other situations, the use of immunotherapy is not indicated. In other patients heparin, aspirin or anticoagulants either alone or associated should be administered depending on each specific case.