Only two cases of juvenile xanthogranuloma of the larynx have been previously reported in the literature. We report a new case, which occurred in an 18-month-old girl. The patient was brought to us for treatment of stridor and respiratory distress. During examination, she was found to have a subglottic mass. The lesion was treated with laser microlaryngoscopy, which relieved the patient's respiratory distress and obviated the need for tracheotomy. Pathologic examination of the mass revealed that it was consistent with a juvenile xanthogranuloma. Juvenile xanthogranuloma is generally a benign and self-limiting disease, but complications can occur when the space that the tumor occupies causes functional impairment.

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