The objectives of the present study were to comprehensively evaluate all the published cases on compound odontoma associated with calcifying odontogenic cyst (COaCOC) in the English literature and to describe the clinical, imaging and therapeutic variables for this condition. In August 2020, an electronic search of the PubMed / MEDLINE, Web of Science, ScienceDirect, Springer, and Scopus databases was carried out. The eligibility criteria included publications with enough information to confirm the diagnosis. Furthermore, we present a clinical case of a 16-year-old male patient with OCCaC, who was treated with enucleation, obtaining favorable and functional results. A total of 32 cases reported in the literature that met the inclusion and exclusion criteria, including ours, were analyzed and discussed. The mayority of the patients were women (n = 17) with an average age of 14.4 years, the maxilla was the most affected bone (n = 22) and the maxillary anterior region was the area with the highest number of cases (n = 18), the main clinical presentations were the volume increase (n = 14) and asymptomatic (n = 14). The choice treatment was enucleation (n = 26) and, in most cases, no recurrence was reported (n = 20). This study allows to update the characteristics of the OCCaC, giving an effective vision of how to treat this rare pathological association made up of two conditions that are completely different from each other.