Inflammatory myofibroblastic tumor (IMT) is a rare lesion, representing 0.04-1.2% of all lung tumors. Brunn first described it in 1939, but its etiology remains uncertain. A 16-year-old patient was admitted to our hospital for further examination following abnormal radiological findings. The physical examination showed no abnormality, and routine hematological and biochemical parameters were within normal range. Chest radiograph revealed homogenous opacity of the right upper lobe with regular margins. Thoracic CT showed a nodular lesion, 30×26 mm in dimensions, with lobular contours in the right hilar. Bronchoscopic examination showed a vascular endobronchial lesion in the anterior right upper lobe, with bleeding when palpated. She underwent right thoracotomy for diagnostic and therapeutic purposes since bronchoscopic biopsy failed because of bleeding. With a pathological diagnosis of IMT, the present report discusses her case accompanied by relevant literature as it is a very rare type of lung tumor. IMT is a rare benign tumor. The diagnosis is difficult to make before surgery since its clinical and radiological features are variable and nonspecific. Although it is a benign lesion, it should be completely resected and patients should be closely monitored following the resection in order to avoid local invasion and recurrence.