Ten consecutive patients who presented initially with adult respiratory distress syndrome, and who did not respond to conventional treatment, underwent open lung biopsy early in the course of their illness. Idiopathic pulmonary fibrosis was diagnosed and aggressive treatment with methylprednisolone sodium succinate and nutritional support was initiated. Eight patients survived and have not required long-term corticosteroid therapy. Previous reports have documented a mortality of greater than 85% with this particular syndrome of idiopathic pulmonary fibrosis in adult respiratory distress syndrome.