Xanthogranuloma of the external auditory canal-an atypical anatomical manifestation.

Juvenile xanthogranuloma is a proliferative cutaneous manifestation encountered in the paediatric population. Adult cases are uncommon, but have been reported. Lesions are prevalent in the head and neck region, but rarely observed in the external auditory canal. We present the case of a 39-year-old female with a rapidly progressing obstructive soft tissue lesion of the external auditory canal. Surgical excision diagnosed the lesion as a rarely observed otological manifestation of juvenile xanthogranuloma. Surgical excision was curative with no locoregional recurrence. Otolaryngologists should consider juvenile xanthogranuloma as a differential for atypical soft tissue cutaneous lesions of the head and neck, including in divergent populations.