Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor of the jaw with a particular histology and a deceptive behavior. The clinical presentation may be misleading, that is why the contribution of incisional biopsy, molecular biology, immunohistochemistry and cytogenetics is essential in the diagnostic process.This review article aims to discuss its various aspects, summarizing the currently published studies of CCOC to enhance the diagnosis and to present a case report of a 41-year-old man that presented to the Department of Maxillofacial Surgery at Gemelli University Hospital in Rome, Italy.
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