Inflammatory myofibroblastic tumor is a rare and distinctive lesion composed of myofibroblastic cells accompanied by an inflammatory infiltration of plasma cells, lymphocytes, and eosinophils that mainly affects viscera and soft tissues of children and young adults. It clinically manifests as a mass with rapid development that may occur at almost any site of the body, but predominantly in the lungs or the upper respiratory tract. It rarely arises in the oral cavity with approximately 15 cases reported in that location. We describe a case of an inflammatory myofibroblastic tumor of the tongue, confirmed by both histopathologic and immunohistochemical analysis, occurring in a 14-year-old boy that spontaneously regressed after the surgical procedures for its diagnosis. Because of some aggressive clinical, histological, and radiological features, this lesion may be confused with a malignant tumor. Therefore, its correct recognition is important to avoid unnecessary extensive and radical therapeutic approaches.