Abstract： Objective To explore the diagnosis and treatment of congenital pulmonary sequestration in children.Methods Between March 2001 and March 2011,total 20 children with congenital pulmonary sequestration underwent treatment in our center,in which 11 were male and 9 were female.Their average ages was 5.3 years (ranging from 4 days to 9 years old) and mean weight was 11.3 kg (ranging from 4 to 28 kg).Their clinical data were reviewed in this study.All cases were examined by chest radiography,and 15 cases of them were further examined by CT.9 cases were misdiagnosed as lung cyst,2 cases as lung tumor,4 cases missed diagnosis and confirmed during surgery.Other 5 cases were examined by 64-slice spiral CT angiography combined three dimensional reconstruction.Abnormal feeding arteries were discovered in 4 case of them and 1 case was highly suspected.Results All cases underwent surgery successfully and postoperative pathology confirmed of pulmonary sequestration.During the operation,we found aberrant supporting arteries from the general circulation to pulmonary sequestration.All cases recover well without recurrence.Conclusions Pulmonary sequestration is rare Their symptoms are non-specific characteristic and are easily to be misdiagnosed or miss diagnosis.Spiral CT angiography combined three dimensional reconstruction is effective way for preoperative diagnosis.The key of surgery is to ligate and cut the supporting arteries from general circulation accurately.