Diagnosis of antiphospholipid syndrome (APS) should be considered in all patients with recurrent systemic or ocular thrombosis in the absence of known risk factors. Because of the frequent ocular involvement in APS patients (as many as 80%), an ophthalmologic assessment should become a routine part of the clinical work-up of all patients in whom APS is highly suspected. The presence of isolated ocular thrombosis with persistently increased titers of antiphospholipid antibodies should be considered as definite APS. Ocular involvement in APS is frequently associated with other manifestations of the central nervous system (CNS), such as transient ischemic attack or cerebral vascular events. Diagnostic tools are needed to better establish a diagnosis of retinal vascular thrombosis. The treatment of isolated ocular APS should be based on the same principles as in all patients with systemic APS. Anticoagulation is aimed to prevent recurrent ocular or cerebral thromboses.