Odontogenic lesions composed of two or more separate odontogenic cysts are uncommon. The calcifying odontogenic cyst (COC) and odontogenic keratocyst (OKC) are odontogenic cysts that exhibit some neoplastic features. Concurrence of these lesions is extremely rare. We report a rare case of COC combined with OKC in a patient and review the relevant literature systemically. METHODS In this article, we present a case report of an 18-year-old boy patient with no medical history whose chief complaint was facial asymmetry. A painless swelling in the right side of the posterior mandible was observed. Histopathological and radiographic investigations revealed a combined odontogenic lesion composed of inflamed OKC with COC. Long-lasting debates on the true nature of OKC and COC, whether cystic or neoplastic, has been existed so far. Their genetic-derived mechanisms can justify the concurrent occurrence of these cysts in a patient. The treatment plan for this lesion included decompression followed by enucleation, curettage, and application of Carnoy's solution. CONCLUSIONS We reported a rare case of an inflamed OKC with COC-like features. Based on the literature, the coincidence of these two odontogenic cysts has been reported for the third time here.