A patient with ulcerative colitis had extensive upper zone pulmonary disease while taking sulfasalazine. Pulmonary function tests showed progressive restrictive and obstructive disease. Lung biopsy showed bronchiolitis obliterans and chronic interstitial pneumonia or fibrosing alveolitis with a mild eosinophilic infiltrate. The patient improved after receiving steroid therapy. A review of the literature of lung disease related to ulcerative colitis and sulfasalazine is presented.