The authors report three patients with systemic lupus erythematosus (S.L.E.) who developed primary retinal vasculitis. All patients showed evidence of four American Rheumatism Association diagnostic criteria for S.L.E. The first case consisted of a woman with retinal central vein occlusion. Auto-immunization against retinal S antigen was present. She improved under heparin therapy. In the second case, a young girl had chronic optic disk vasculitis for seven years. In the third case, retinal arterial occlusive disease was present with optic nerve involvement. The differential diagnosis with multiple sclerosis is discussed. S.L.E. is considered as autoimmune disease where circulating immune complexes play a major role in the pathogenesis. The ocular complications are probably a manifestation of the widespread systemic disease in patients without arterial hypertension. The authors summarize the ocular findings in S.L.E.: venous obstruction, constriction of retinal arteries, capillary vasculitis and disc neuritis. In some cases, there may be the first manifestations of the disease.