【Abstract】Objective To Investigate the clinical manifestations, diagnosis and treatment of pulmonary sequestration. Methods 30 patients with pulmonary sequestration confirmed by histopathological studies were analyzed and related literatures were reviewed. Results Among these patients, 25 were intralobar type and 5 were extralobar type. All patients with intralobar type had symptoms of cough, expectoration, fever and hemoptysis while 5 patients with extralobar type were asymptomatic. 20 patients were diagnosed preoperatively by chest radiography, plain CT scan, enhanced CT scan or CT angiography. Operation was performed in all the patients, with successfully recovery after operation, and no relapse was found. Conclusions The clinical manifestations of pulmonary sequestration are nonspecific. Although invasive, Selective arteriography was gold standard for pulmonary sequestration. Noninvasive angiography including enhanced CT scan, CT angiography have become important supplements to selective arteriography. Surgery is the optimal treatment, while the effectiveness of interventional therapy needs further investigations.