Macrolide antibiotics have been shown to have a beneficial effect in a number of pulmonary conditions that are characterized by inflammation, including cystic fibrosis, asthma, and cryptogenic organizing pneumonia. We report the first case of desquamative interstitial pneumonia (DIP) showing a favorable response to treatment with clarithromycin. If confirmed, this observation would add DIP to the list of pulmonary disorders that are amenable to the beneficial antiinflammatory effects of macrolides.