BACKGROUND: Systemic lupus erythematosus (SLE) is a multisystemic inflammatory disease of unknown etiology. Physiopathology includes small vessel occlusion and auto-antibody production. Neuro-ophthalmic complications can be detected in 10-30% of SLE cases. Further, some patients can present with neuro-ophthalmic complications prior to diagnosis of SLE. PATIENTS AND METHODS: We report seven cases of neuro-ophthalmic complications of SLE demonstrating lesions from the retina to the brain. The other possible manifestations of SLE will be reviewed. RESULTS: Two cases were not yet diagnosed with SLE when neuro-ophthalmic disease occurred. Anti-double stranded DNA antibodies were detected at a very high titer, suggesting SLE, later confirmed by rheumatologists. CONCLUSIONS: Ophthalmologists should be aware of the unusual patient presenting with a neuroophthalmic disorder prior to the diagnosis of SLE. In such cases, autoantibodies should be sought. Antinuclear antibodies are very sensitive but non specific for SLE. Anti-double stranded DNA antibodies are specific for SLE and elevated titer should raise the suspicion of undiagnosed SLE.