Clear cell odontogenic carcinoma of maxilla: A diagnostic challenge

Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor which occurs mostly in the mandible. It is primarily seen in fifth to seventh decades with a female predilection. We report a case of CCOC in the maxillary arch of a 66-year-old woman. Morphologic examination along with histochemical and immunohistochemical markers led to the establishment of the diagnosis. It is important to diagnose this entity and differentiate it from other clear cell tumors in the head and neck region as it is a locally aggressive tumor with a propensity for regional, nodal, and distant metastasis.