Background: We aim to present an unique case of inflammatory myofibroblastic tumor (IMT) of the brain parenchyma and study the clinical presentation, imagine characteristic, intraoperative findings, and histopathology features of IMT in the brain parenchyma. Case Presentation: A 36-year-old female with IMT come to see doctor presented with a 3-month history of progressively worsening weakness in the left upper limb along with alalia. Serial magnetic resonance imaging studies revealed a lesion within the right frontal lobe. The lesions were locally clear boundary with peripheral tissues. A right frontotemporal craniotomy was performed and the specimens were detected by immunohistochemical staining and light microscopy. Through the microscope, the tumor was composed of large number of chronic inflammatory cells and spindle cells. From immunohistochemical stains, it demonstrated CD34 (+), SMA (+), Vimentin (+), and actin (+) were positive meanwhile S-100(-) was negative. Conclusion: IMT of the right frontal lobe is a rare lesions. Surgical resection is the best and the most effective treatment.