A case of transverse myelopathy in systemic lupus erythematosus with subacute onset and fatal course is reported. A review of the literature is done and clinical, laboratorial, therapeutics and pathological aspects are discussed. The authors call attention to the poor results of therapy and discuss the participation of immunological factors leaving to demyelination which are, aside the classical vascular involvment, the probable mechanisms in the neurological manifestations of systemic lupus erythematosus. The authors believe that, in their case, such a demyelinative mechanism was responsable for the clinical picture, although a necroscopic examination was not allowed.