Clear cell odontogenic carcinoma (CCOC) is histologically characterized by solid structures of clear cells. Clear cell ameloblastoma (CCAM) is histologically characterized by an ameloblastomatous component intermixed with an extensive clear cell component. In all literature reviews no separation has been made between the clinicopathologic features of CCOC and CCAM. The purpose of the present study was to review and analyze the clinicopathologic and radiologic features and the biologic behavior of CCOC and to compare them to those of CCAM in order to evaluate the possible separation between the two lesions. A Medline search of the English language literature was carried out for CCOC and CCAM. Cases were classified according to their histologic features. A total of 27 cases of CCOC ( 26 from the literature and one new case) and 8 cases of CCAM were found. CCOC patients demonstrated a M:F=1:2.4, with a mean age of 59 years. CCAM patients demonstrated a M:F=1.7:1, with a mean age of 44 years. CCOC was almost equally distributed between the anterior and posterior regions of the mandible, while CCAM was more common in the anterior region. Several patients with CCOC presented with metastases at time of diagnosis, while patients with CCAM usually developed metastases only after several recurrences. Mortality rate was considerably higher among CCAM compared to CCOC patients, 38% and 13%, respectively. Differences in the histologic features, age distribution, gender, location and biologic behavior of CCOC and CCAM suggest that these lesions could represent two different entities.