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Background  Schwannomas are uncommon tumors of the external auditory canal. In the English literature, very few cases of schwannomas originating in the external auditory canal were reported and none of them showed chondroid metaplasia. We report the first case of schwannoma with chondroid metaplasia in this location.    Case presentation  In this report, we described a 22-year-old white man who presented with an external auditory slow growing mass. A computed tomography scan of the temporal bone demonstrated a well-circumscribed, soft tissue mass narrowing most of the external auditory canal. A surgical biopsy was performed and the histological examination showed a schwannoma with chondroid metaplasia.    Conclusion  Schwannoma should be considered in the differential diagnosis of benign or malignant tissue masses involving the external ear canal.

Schwannoma with chondroid metaplasia of the external auditory canal – a rare finding in a rare location: a case report