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Inflammatory myofibroblastic tumor of the airway is a very uncommon benign primary neoplasm in pediatric age group with increased local recurrence rate and potential metastatic spread. We describe a case of a 6-year boy who was brought to the pediatric emergency with severe respiratory distress, dry cough, and stridor. Contrast-enhanced computed tomography and magnetic resonance imaging (MRI) of the neck showed a polypoidal mass lesion in the right anterolateral trachea causing significant airway narrowing. Bronchoscopic findings correlated with the imaging. The lesion was confirmed at surgery and was completely removed by surgical excision. Histopathology revealed an inflammatory myofibroblastic tumor. MRI findings of this entity in a child have not been reported before.