Background: Clear cell odontogenic tumour was first described by Hansen (1985). In 1992, World Health Organisation (WHO) classified the lesion as a tumour and due to its aggressive nature (local recurrence, regional lymph node and distant metastasis), was formally classified as malignant in 2005. Less than 100 cases have been reported in the English literature as of 2016. Case description: We present the case of a 43 year old female, with an expansile intra-oral swelling in the anterior mandible. The case has been discussed from histopathological perspectives of differentials and cues to diagnosis. The patient in the current case was lost to follow up subsequent to the diagnostic biopsy. Conclusion: It is imperative that an oral pathologist differentiates the CCOC from other histopathologically similar lesions such as the clear cell variant of CEOT as well as non-odontogenic lesions like the clear cell variant of mucoepidermoid carcinoma and Clear cell carcinoma of salivary glands. However, one must be cognizant of the aggressive nature of CCOC with significant rates of recurrence and distant metastases thereby warranting its consideration in the list of differentials of jaw tumours with a conspicuous, albeit non-pathognomonic clear cell change.
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