Bilateral central retinal artery occlusion (CRAO) has been rarely reported as the primary manifestation in patients with systemic lupus erythematosus (SLE).The severe retinal vaso-occlusive diseases usually cause devastating and permanent damage to visual function in spite of vigorous treatment.A 42-year-old Chinese woman presented with abrupt bilateral vision loss.The diagnosis of bilateral CRAO was suggested by the ocular presentation and fluorescein angiography.Laboratory studies showed positive results of antinuclear antibody,anti-Ro/SSA anti-La/SSB; decreased levels of C3,C4 complement and normal levels of antiphospholipides antibodies (APAs).Her visual acuity deteriorated despite systemic steroid and immunosuppressant treatment.Severe vaso-occlusive retinopathy may be an earlier manifestation of SLE without elevated level of APAs.