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Objectives: Inflammatory myofibroblastic tumor (IMT) is a rare benign lesion, especially in the pediatric age. There are several cases described in pulmonary, digestive and renal localizations, but involvement in head and neck area is infrequent. Methods: Case report and review of the literature. Results: A 1 year and 11 months old child, during 2 months had clinical signs of nasal respiratory insufficiency and epistaxis subsequently developing a purulent rhinorrhea and a sleep apnea. His pediatrician previously requested a sinus and cavum X-ray with the finding of an image compatible with an intranasal mass. Endoscopic resection was performed of the mass with further immunohistochemical analysis showing the result of a lesion compatible with IMT. Conclusions: Even though there are very few cases in scientific literature of a tumor with these characteristics in infants, IMT must be present as a differential diagnosis of intranasal masses. The role of the pathologist is essential to reach the definitive diagnosis and the performance of an early surgical treatment decreases aesthetic consequences in this pathology.