Inflammatory myofibroblastic tumors of the lung are rare in adults but common among pediatric lung tumors. A 4-year-old girl was evaluated for respiratory symptoms, dysphagia, and weight loss. Radiology revealed a right hilar mass that persisted despite antibiotic treatment. On exploration, a hilar mass involving the pulmonary vasculature, diaphragm and gastroesophageal junction was found. Aggressive surgical excision including a right pneumonectomy was performed. Histopathology confirmed an inflammatory myofibroblastic tumor with no features of malignancy. At the 2-year follow-up, the child was clinically stable with no recurrence. Dysphagia is a very rare presentation of such tumors.
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