Antiphospholipid syndrome (APS) is characterized by increased hypercoagulability and divergent symptoms including ocular manifestations. In APS patients arterial and/or venous thromboses and repeated fetal loss are diagnosed in presence of antiphospholipid (aPL) antibodies. Antiphospholipid antibodies are heterogeneous group of immunoglobulins with different antigenic structure. Primary APS is defined in the absence of underlying disease, while secondary APS is seen within another pathological condition. In both primary and secondary APS ocular and neuroophthalmic manifestations, such as retinal arteritis, retinal venous occlusion, ischemic optic neuropathy, transient loss of vision - amaurosis fugax, diplopia and others can be diagnosed. In secondary APS occlusion of central retinal artery and vein (OACR, OVCR) is the most common finding, thus when found in younger patients it should be considered indicative of APS. Bilateral ocular changes are considered more significant since they affect both ocular function and life prognosis.