Inflammatory myofibroblastic tumors are regarded as intermediate-grade tumors with a potential for recurrence. Although these lesions have been found in nearly every anatomic location, there are few documented cases of esophageal localization. The rare case reported here concerns a 55-year-old woman with an extremely large inflammatory myofibroblastic tumor of the esophagus; the tumor was 20 cm in length and 6 cm in diameter. Wide surgical excision was performed. Histopathologic and immunohistochemical analyses confirmed the diagnosis of an inflammatory myofibroblastic tumor. There was no recurrence or tumor metastasis 6 months after the operation. (Ann Thorac Surg 2010; 89: 607-10) (C) 2010 by The Society of Thoracic Surgeons