Cystic fibrosis (CF) has a variety of pulmonary manifestations that include pneumonia, pulmonary abscess, bronchiectasis, hemoptysis, and pneumothorax. Although newer therapies have greatly improved survival of patients with CF, surgical interventions for definitive treatment of these sequelae are often required. Several reports have illustrated that, with the current advances in the perioperative treatment and care of CF patients, surgical interventions for these pulmonary manifestations may be performed safely, resulting in a greatly improved quality of life. Also, although improvements in lung transplantation offer new hope for definitive treatment of those patients with cystic fibrosis, special considerations regarding other surgical issues, such as the prevalence of gastroesophageal reflux, need to be addressed. © 2008 Elsevier Inc. All rights reserved.