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Juxtaoral organ of Chievitz presenting clinically as a tumour

An extremely rare hamartomatous lesion of the juxtaoral organ of Chievitz (JOOC) in a 63 year old man is reported. The tumour appeared as a large mass in the infratemporal fossa with associated mandibular bone resorption; histologically, it was well encapsulated and composed of numerous tangled masses of benign squamous epithelial nests and mature fibrofatty tissue. There were no histological features suggestive of neoplastic transformation. A literature survey confirmed that this is the first adult case of JOOC presenting clinically as an extraoral tumour.