Juvenile xanthogranuloma (JXG) is a benign, spontaneously regressing lesion that usually occurs during the first year of life, but may also occur in adulthood. Although the most common presentation of JXG is the cutaneous lesion, it can also manifest in various visceral organs. JXG of the external auditory canal is extremely rare, and there have been only a few reports of those cases in the English literature. In this study, we present a case of pathologically proven JXG that occurred in the external auditory canal with a symptomatic clinical presentation.
