A calcifying cystic odontogenic tumor (CCOT) is a proliferation of odontogenic epithelium and scattered nests of ghost cells and calcifications that may form the lining of a cyst, or present as a solid mass. It was previously described by Gorlin et al in 1962 as a calcifying odontogenic cyst. Dentigerous cysts are developmental odontogenic jaw cysts, commonly manifesting in the second and third decades of life. The present study reports an asymptomatic case in a 13-year-old boy who was referred to the outpatient clinic of the Osaka Dental University Hospital (Osaka, Japan) for additional investigation of an area of radiolucency in the lower right jaw. X-ray demonstrated a unilocular, well-circumscribed, radiolucent lesion in the mandible, which measured 30x20 mm, with radiopaque structures within it. Enucleation of the lesion with tooth extraction was performed, which histopathologically revealed features of a CCOT and a cyst. To the best of our knowledge, the occurrence of such a lesion has not been previously identified. The present study examined the significance of the case with a brief review of the literature.