A 70-year-old man in whom nodular and reticular shadows had been noted on chest radiography since 1992 was admitted to our hospital with complaints of persistent cough and dyspnea on exertion in August, 2000. The definitive diagnosis of lung abnormalities was not confirmed by TBLB. He was re-admitted to our hospital to undergo a lung biopsy by video-assisted thoracoscopic surgery. Although desquamative interstitial pneumonia was diagnosed, respiratory failure developed rapidly after surgery. He responded well to high-dose steroid administration followed by maintenance therapy with a moderate dose of steroid, resulting in a considerable importance of the clinical condition associated with a significant decrease in the ground-glass opacities and infiltrative shadows. Although we could find no literature reporting acute exacerbation of DIP, our case demonstrates that DIP may also be acutely exacerbated when a severe insult is superimposed.