Background: Postpneumonectomy syndrome (PPS) is a rare complication after pneumonectomy. It consists of an excessive mediastinal shift resulting in compression and stretching of the tracheobronchial tree and the esophagus. The aim of this study was to give a comprehensive overview of diagnosis, variety of symptoms and evaluation of surgical treatment of PPS. Methods: We retrospectively reviewed the charts of all our patients with PPS since 1994 with respect to symptomatology, treatment and outcome. Our results were compared with case reports and case series in the literature. Results: Six women with a median age of 56.5 years (range 49-65) developed PPS after pneumonectomy for the treatment of lung cancer. Four presented with a right PPS and two with a left PPS, respectively. Symptoms consisted of shortness of breath in all patients and dysphagia as well as heartburn in two patients. Correction of PPS required re-exploration of the pneumonectomy space, reposition of the mediastinum followed by the insertion of single silicone prosthesis in five patients or fixation of the mediastinum with a xenopericardial graft in one patient. We could observe an improvement of the FEV(1)/FVC ratio in all our patients and the clinical improvement of shortness of breath was better than we expected by changes of lung function. Four patients returned to their regular activities with a follow-up of four years. We found 73 cases of PPS in the literature, on the right side in 50 patients (68%) and on the left side in 23 patients (32%). Fifty-nine patients (81%) were treated surgically. Symptoms can be suspicious for cardiogenic origin and vary from heartburn to recurrent syncopes. Conclusion: PPS is rare and not predictable. It can occur after right or left pneumonectomy. Symptoms are manifold and result from a shift, leading to compression and stretching of the two conduits located within the mediastinum, the tracheobronchial tree and the esophagus and consists of shortness of breath, stridor and heartburn. Diagnosis must be made by exclusion. Implantation of prosthesis is the most commonly used and effective treatment.