Systemic lupus erythematosus(SLE) is the prototypic immune complex disease which involves various organs. Chronic interstitial cystitis and protein-losing enteropathy are uncommon manifestations of SLE and have good response to steroid. Only one case of SLE associated with chronic interstitial cystitis and protein-losing enteropathy has been reported which was resistant to steroid treatment but dramatically responded to cyclophosphamide. We describe a case of a female patient who developed systemic lupus erythematosus associated with chylothorax, interstitial cystitis and protein-losing enteropathy with literature review. This case was initially resistant to steroid but with the addition of cyclophosphamide, chylothorax, chronic interstitial cystitis and protein-losing enteropathy markedly improved. This is the first case of SLE that developed chylothorax as well as chronic interstitial cystitis and protein-losing enteropathy.(Korean J Med 59:555-560, 2000)