Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that involves various organs. Surgery is the mainstay of therapy for this tumor. Approximately half of all IMT cases have anaplastic lymphoma kinase (ALK) rearrangements; therefore, the ALK inhibitor crizotinib is suggested as a promising treatment for unresectable cases with ALK rearrangements. In cases withoutALK rearrangement, chemotherapy is an alternative treatment for unresectable tumors.