ObjectiveTo explore the clinical characteristics, diagnosis and treatment of thoracic inflammatory myofibroblastic tumor.MethodsThe clinical data, pathological features, treatment and prognosis from 10 patients with thoracic inflammatory myofibroblastic tumor confirmed by pathology were analysed retrospectively from April 2012 to April 2019 at Jiangsu Province Hospital (The First Affiliated Hospital of Nanjing Medical University).ResultsTen participants including six males and four females with a mean age of 37.9 years old. Lesions were detected by physical examination in five patients, cough, chest pain and hemoptysis were the common symptoms. A total of 10 lesions including six in the right lung, three in the left lung and one in the mediastinum. Nine patients were treated with surgery, and one patient received high-frequency electrocautery though rigid bronchoscopy under general anesthesia. All the patients were confirmed by immunohistochemistry, positive rate of smooth muscle actin was 70%, positive rate of anaplastic lymphoma kinase was 70%. The mean follow-up time was 35.9 months, and one patient relapsed the other nine patients were cured.ConclusionsInflammatory myofibroblastic tumor is potentially malignant or low malignant, the clinical manifestations and imaging findings are not specific, once confirmed by pathology, radical surgery is the first choice. For the lesion limited to the airway, interventional therapy could be the choice, but close follow up is needed.