Abstract: Objective To summarize the clinical experiences and surgical treatment of pulmonary sequestration (PS) in order to improve the diagnosis and treatment of PS. Methods Between August 1993 and February 2007, our department enrolled 21 PS patients, 8 male patients and 13 female patients, with the age ranging from 13 to 70 years old. The patients were examined by chest radiography, computerized tomography (CT), computerized tomography angiography (CTA), magnetic resonance imaging (MRI), position emission tomographyCT(PET-CT) before the surgery. Sequestrectomy was performed on patients with extralobar sequestration (ELS) and lobectomy was performed on patients with intralobar sequestration (ILS). There were 10 cases of left lower lobectomy, 3 cases of right lower lobectomy, 4 cases of left sequestrectomy, 3 cases of right sequestrectomy and 1 case of total pneumonectomy. Results Postoperative pathology confirmed all cases of PS, including 7 cases of ELS and 14 cases of ILS. Seven patients were diagnosed to have PS by preoperative diagnostic procedures. During the surgery, we found aberrant supporting arteries from the general circulation in 18 cases among which 11 were supported by the thoracic aorta, 6 by the abdominal aorta and 1 by both the thoracic and abdominal aorta. The diameter of the aberrant artery was between 0.2 cm and 1.1 cm (mean 0.7 cm). Double ligation and transfixion were performed during the operation. In addition, we found venous drainage through the inferior pulmonary vein in 3 patients and double ligation was performed. No perioperative death or complications occurred. Followup was done till January 2009 on all the patients but one with a followup rate of 95.2% (20/21). The followup time ranged from 12 to 67 months. All patients survived well except that 1 died from liver metastasis 2 years after the operation because of lung cancer. Conclusion PS is rare and its symptoms are nonspecific, which can cause misdiagnosis and missed diagnosis. The diagnosis of PS mainly depends on CT, CTA, MRI and selected arteriography. Once diagnosed, PS should be removed by surgery. During the surgery, aberrant vessels should be separated and treated with double ligation and transfixion. As for those big aberrant vessels, transfixion can be performed after vascular decompression.