Cystic fibrosis (CF) is a multisystem disorder characterized by progressive lung disease. Life expectancy is, however, continually improving. Patients with CF will therefore present with an increasing number of conditions, some of which will require operative management. We present our experience of the management of a patient with CF who underwent a subtotal esophagectomy for adenocarcinoma of the esophagus. No significant difficulties were encountered in the perioperative management of the patient. Despite a decline in his lung function and weight postoperatively, he remains clinically stable. Major surgery can be successfully undertaken in selected patients with CF.