Odontogenic cysts showing features of two or more distinct types of cysts are rare. Particularly, calcifying odontogenic cyst (COC) associated with odontogenic keratocyst (OKC)-like areas have been not reported. The patient, an 81-year-old man, was referred for diagnosis of a mandibular radiolucent lesion of unknown duration. On cone-beam computed tomography, sagittal views revealed a well-delimited, unilocular, and hypodense lesion located in the right edentulous mandibular body, which caused enlargement of the buccal and lingual cortical bone. Panoramic reconstruction revealed that there was thinning of the superior cortical bone. Residual radicular cyst was the clinic-radiographic diagnosis. Under local anesthesia, an incisional biopsy was performed. Microscopically, a cystic cavity lined by ghost cells was observed, with basal cells showing reverse nucleus polarity. Solid areas were also noted. Moreover, there were cystic cavities lined by epithelium presenting a corrugated parakeratin surface and prominent basal cells disposed in a palisaded fashion. Cytokeratins 14 and 19 were positive in both areas, COC and OKC. However, bcl-2 was positive only in COC areas. Thus, the diagnosis was of COC with OKC-like areas. The lesion was excised, confirming these histopathological findings. Currently, the patient is under follow-up, without clinical or imaging signs of recurrence after 2 years of treatment. In conclusion, COC with OKC-like areas is rare and this association seems present a good prognosis.