Abstract: Objective To explore the etiology, diagnosis, treatment and prognosis of inflammatory myofibroblastoma (IMT)and improve understanding of this disease. Methods Clinical and imaging characteristics of an IMT patient with simultaneous involvement of respiratory and digestive system in Chinese PLA General Hospital in 2018 were analyzed and related literatures were reviewed. Results The patient was a 53-year-old male and mainly presented with dry cough and wheezing after exercise.Bronchoscopic examination in local hospital showed a neoplasm in left main bronchus and lung CT showed left lung atelectasis.Then he was referred to Chinese PLA General Hospital for further treatment. The patient had undergone surgery because of esophageal tumor in other hospital in October, 2017, and diagnosed as inf l ammatory fi broid polyp. However, pathology consultation from our hospital considered it as inflammatory myofibroblastoma (low malignant potential). Interventional therapy such as bronchoscopic argon plasma coagulation (APC) and high frequency electric knife resection were given. The clinical symptoms were improved obviously after operation. The imaging examination showed that the left lung was dilated. No serious complication occurred during the treatment. Postoperative pathological result was in accordance with inflammatory myofibroblastic tumor. Conclusion Inf l ammatory myofibroblastoma is a rare low-grade malignant tumor, especially in patients with simultaneous bronchial and esophageal involvement. The definitive diagnosis depends on histopathological examination, and surgery is the fi rst choice. If the patient has no chance of operation, such clinical manifestations as airway obstruction and pulmonary atelectasis can be treated by endoscopic intervention combined with radiotherapy and chemotherapy.