Inflammatory myofibroblastic tumor (IMT) is a rare lesion primarily found in the lungs.This tumor has the potential for local recurrence and a low likelihood of metastasis. Histologic morphology can reveal the infiltration of inflammatory cells in a myofibroti background with anaplastic lymphoma kinase (ALK) overexpression. In the article, we present a case of a 59-year-old male with a well-circumscribed exophytic tumor approximately 2.5 cm in diameter, located in the midline of the dorsal tongue. Initial biopsy showed only granulation tissue without definite diagnosis. However, typical histopathological features of IMT were ultimately identified in the specimen obtained from complete surgical excision. The purpose of the paper is to present a case of oral IMT, which demonstrates a change in both clinical appearance and histopathology after initial biopsy and review the literature on potential causative factors and histologic patterns. (J Taiwan Otolaryngol Head Neck Surg 2023; 58:111-115)