Objective: The aim of this study was to describe the anesthetic management and perioperative outcomes in patients with Swyer-James syndrome (SJS), a rare acquired pulmonary disorder caused by postinfectious bronchiolitis obliterans resulting in airway obstruction and focal areas of emphysema or bronchiectasis.Design: A retrospective computerized search of the medical records database at a large academic tertiary referral center was performed for patients with SJS from January 1, 2001 through July 31, 2012 who underwent procedures requiring anesthesia. A review of the perioperative course in the SJS patients as well as in those identified by a literature search was performed.Setting: Academic tertiary referral center, both inpatient and outpatient settings.Participants: Patients with a diagnosis of SJS.Interventions: No interventions were performed.Measurements and Main Results: The authors identified 4 patients with SJS who underwent 7 uneventful anesthetics including one lung resection. Three patients were symptomatic preoperatively. The literature review identified 23 SJS patients who underwent lung resection for recurrent pulmonary symptoms. One patient developed hypoxemia during one-lung ventilation. Three patients had a prolonged hospital stay (β₯10 days). All patients undergoing lung resection were young (β€42 years of age).Conclusion: Though SJS may be an incidental finding, these patients may have marked symptomatology, recurrent pulmonary infections, and pneumothoraces that may require lung resection. The patients tolerated anesthesia well. In severe SJS cases, pulmonary pathology and perioperative management strategies parallel that of patients with severe obstructive pulmonary disease.