Antiphospholipid syndrome is a rare cause of ocular vaso-occlusive disease, but is associated with significant systemic morbidity and mortality. Thus, early diagnosis and treatment is essential.Although the pathophysiology of antiphospholipid syndrome continues to be poorly understood, there has been continued progress with regard to the relationship between antiphospholipid antibody and its target, β-2-glycoprotein I. Due to numerous limitations with standard serologic evaluation, new approaches to the evaluation of patient serum are being considered. New guidelines for the treatment and management of antiphospholipid antibody syndrome have been established by the 13th International Committee on Antiphospholipid Antibodies.A better understanding of the pathophysiology behind antiphospholipid antibody syndrome has led to novel approaches in the diagnosis and treatment of this disease.