We report a rare case of bilateral macular infarction as an ocular presenting sign of systemic lupus erythematosus (SLE). A 29-year-old woman presented to our ophthalmologic clinic with a 1-week history of progressive visual loss in her left eye after she had visited a rheumatologic clinic where SLE was diagnosed. At examination, best-corrected visual acuity (BCVA) of the right eye was 6/6, and for the left was counting fingers. Fundus examination revealed perivascular hard exudates along some branches of vessels in both eyes. After pulse therapy, her BCVA in the right eye declined to 6/30 and in the left improved to 3/60. She was administered sub-Tenon's injections of triamcinolone acetonide 50 mg/week in both eyes for 3 weeks. Her BCVA improved to 3/6 in her right eye and remained at 3/60 in her left eye. Macular infarction is an uncommon but most severe complication of SLE. Early and regular exam of the fundus in patients with SLE is necessary to avoid progression of severe ocular complications.