A nine-month-old boy with a large perauricular nodular mass extending
into the external auditory canal was seen. The mass was present since
birth and was responsible for conductive hearing loss. Light microscop
y and immunohistochemical studies were consistent with juvenile xantho
granuloma. Initiation of local and systemic corticosteroid therapy was
followed within two weeks by complete regression of the mass and full
recovery of hearing. The clinical features, histologic findings, and
differential diagnosis of this condition are discussed.