Retinopathy in systemic lupus erythematosus generally consists of cotton-wool spots with or without intraretinal hemorrhages. Although rare, a more severe retinal vaso-occlusive disease, termed retinal vasculitis, has been described. We report data from 11 patients with SLE and severe retinal vaso-occlusive disease. Visual outcome was often poor, with 55% of the involved eyes suffering visual loss, frequently to an acuity of worse than 20/200. The systemic feature significantly associated with severe retinal vascular disease was central nervous system involvement (CNS lupus), present in eight (73%) of the 11 patients vs an overall prevalence of 37%. The association of CNS lupus with severe retinal vaso-occlusive disease probably reflects a similar pathogenetic mechanism and microangiopathy.