The calcifying odontogenic cyst (COC) has been reported to be associated with odontoma in about 24% of cases. Separation of the cases of COC associated with odontoma (COCaO) may lead to a better understanding of the pathogenesis of this lesion. A screen of the literature revealed 52 cases of COCaO. The male to female ratio was 1:1.9, with a mean age of 16 years. The most common location was the maxilla (61.5%). The radiographic appearance of most cases (80.5%) was of a well-defined, mixed radiolucent-radiopaque lesion. Histologically, the lesions usually consisted of a single large cyst (which is similar to simple COC) with tooth-like structures that appear to be an integral part of the lesion, giving the impression of a single lesion. In addition to the unique histologic features, differences in gender and in distribution were found between the cases of COCaO and those of simple COC. COCaO may be regarded as a separate entity and classified as a benign, mixed odontogenic tumor. The term odontocalcifying odontogenic cyst is suggested.