Inflammatory myofibroblastic tumor (IMT) is an uncommon neoplasm that is usually located in the lung in the pediatric population. These tumors contain a variety of cell types with the myofibroblast being dominant. When located in the upper airway, IMTs tend to be less aggressive, but have the potential for local invasion and recurrence. We present an unusual case of IMT in the pediatric larynx and review the medical literature describing the common locations, diagnosis, etiology, histology, and treatment of this tumor. The mainstay of treatment is complete surgical excision. Careful and frequent follow-up including frequent fiberoptic laryngoscopy and CT scans are recommended to evaluate for recurrence. More aggressive resection may be necessary if multiple recurrences occur.
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