We describe a 23-year old Brazilian woman with systemic lupus erythematosus and protein-losing enteropathy. Small intestinal biopsy revealed lymphangiectasia. Protein-losing enteropathy responded to corticosteroid therapy and should be suspected in cases of systemic lupus erythematosus with hypoalbuminemia without proteinuria and liver failure. A review of the English literature is presented with comments on pathogenesis.