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Inflammatory myofibroblastic tumor (IMT), also known as inflammatory pseudotumor, is a benign lesion predominantly found in the lung and abdomen. Sporadic cases have been reported in the trunk, genitourinary tract, and extremities as well as in the head and neck. Of critical importance is this entity's correct histopathologic diagnosis that differentiate it from malignant neoplasms such as spindle cell carcinoma and fibrosarcoma, benign tumors such as neurofibroma, and other pseudoneoplastic lesions such as nodular fasciitis. Correct diagnosis is followed by wide local excision to prevent recurrence; however, treatment must be tailored to the location of tumor and the condition of the patient. We present a unique case of IMT of the trachea presenting with acute upper airway obstruction in a pregnant woman. Diagnostic considerations as well as the anesthetic and surgical approach are discussed. (Am J Otolaryngol 2002;23:362-367. Copyright 2002, Elsevier Science (USA). All rights reserved.)