A 50-yr-old woman with chronic ulcerative colitis developed severe restrictive lung disease, which progressed after total colectomy and cessation of sulfasalazine therapy. Open lung biopsy showed severe interstitial fibrosis and bronchiectasis. The patient's condition did not improve with steroid treatment. It is concluded that the patient's lung disease may represent an extracolonic manifestation of ulcerative colitis. A review of the literature is presented.