Objective To investigate the clinical characteristics,causes of misdiagnosis and preventive measures of pulmonary sequestration(PS)with elevated tumor markers.Methods The clinical data of PS 3 patients with elevated tumor markers who had been misdiagnosed as lung cancer were retrospectively analyzed from June 2019 to June 2022,and the rele-vant literature was reviewed.Results Three patients were admitted due to cough and phlegm for 2 weeks,cough with fever for 2 weeks,and a space occupying lesion in the left lower lung for 2 months,respectively.There was no history of recurrent pulmonary infection in the 3 patients.CT examination of chest showed solid mass shadow(in the lower left lung in two patients and in the lower right lung in one patient)and at least one tumor marker was elevated.Only 1 patient underwent enhanced CT examination before operation.All patients were misdiagnosed as lung cancer before operation and underwent surgical resection.The diagnosis was confirmed as PS by pathological examination after operation.At 3 months after the operation,the tumor markers of 3 patients returned to normal.Conclusion PS can lead to the increase of tumor markers,which is more likely to be misdiagnosed as malignant tumor,and should be carefully screened in clinical setting to avoid misdiagnosis.