Juvenile xanthogranuloma (JXG) is a benign, non-Langerhans cell histiocytic lesion that generally affects infants and children. These lesions characteristically appear as a solitary, yellow, cutaneous nodule of the head, neck, or trunk. Subcutaneous and extracutaneous forms can involve the gastrointestinal tract, kidney, lung, gonads, pericardium, central nervous system, temporal bone, larynx, and eye. We describe the clinical presentation, imaging, histochemical findings, and management of a solitary JXG of the tympanic membrane in a 17-month-old girl. The patient underwent surgical resection and was without disease several months following surgery and reconstruction of the defect. To the best of our knowledge, this is the first reported case of a JXG of the tympanic membrane.
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