Odontogenic carcinomas are exceedingly rare. Our knowledge of these tumors is based mainly on data from individual case reports or small case series. Clear cell odontogenic carcinoma (CCOC) is grouped among these lesions. It was originally described in 1985 by Hansen et al1 as an “aggressive tumor” of odontogenic origin. However, lacking evidence of lymph node involvement or distant metastases in their series of 3 cases, these authors were unable to identify it as a carcinoma. An additional 19 cases have since been reported; among which are cases in which lesions recurred and metastasized to regional lymph nodes and distant organs. Jaw lesions with microscopic features that resemble those of CCOC include clear cell variants of ameloblastoma and calcifying epithelial odontogenic tumor (Pindborg tumor), metastatic renal cell carcinoma, and some central salivary gland tumors. We describe a case of a CCOC of the mandible and review previous reports of this uncommon malignant neoplasm.