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Plexiform neurofibroma is a rare benign tumor developed from cells of the sheath of Schwann peripheral nervous system often associated with neurofibromatosis type 1 or Von Recklinghausen disease. Laryngeal involvement in neurofibromatosis is rare and usually presents with obstructive respiratory symptoms. Imaging including CT and MRI provides the diagnosis as well as the staging of this lesion, whose treatment is primarily surgical. We report the case of a plexiform neurofibroma of the larynx found in a 4-year-old child revealing a mass completely obstructing the laryngeal vestibule.
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