A 58 year old man with arthralgia had been treated with antiinflammatory drugs since November, 1987. On March 1988, he was admitted to a local hospital because of jaundice. On April 18, exploratory laparotomy was done to look for the cause of continued jaundice. Macro-pathological and micropathological findings of the liver were compatible with those of intrahepatic cholestasis. On April 29, he was transferred to Kyushu University Hospital because of persistent jaundice. Physical examination showed jaundice, tachypnea and low grade fever. Laboratory examination showed elevated level of serum bilirubin, marked hypoxia and endotoxemia. Plain chest radiogram revealed ground-glass appearance in bilateral lung fields. He was diagnosed to be complicated with adult respiratory distress syndrome (ARDS). Glucocorticoid therapy and oxygen administration were started immediately. But pulmonary fibrosis developed and he died of respiratory failure. Histological findings of lung necropsy specimen were compatible with those of ARDS. These data strongly suggested that endotoxemia and reticuloendothelial dysfunction due to intrahepatic cholestasis had played important roles to develop ARDS.