A case of unilateral central vein occlusion occurring in a normotensive patient with systemic lupus erythematosus (SLE) is reported. This vasculitis is related to SLE. In spite of the observation of circulating immune complex and a positive response to the human basophil degranulation test in the presence of bovine soluble retinal antigen, the real pathogenesis of this unilateral vasculitis remains obscure.