Several visual disturbances have been associated with the presence of antiphospholipid antibodies, including vaso-occlusive retinopathy and ischaemic optic neuropathy. The latter has been rarely described in patients with "primary" antiphospholipid syndrome, and, when present, is generally associated with neurological disorders. We describe the case of a patient presenting with optic neuropathy in whom a "primary" antiphospholipid syndrome was diagnosed, and review all previously reported similar cases. The pathogenesis and treatment are also discussed.