The calcifying odontogenic cyst (COC) is a rare lesion that involves the jaws which, at times, may evolve into a solid, benign, tumor-like mass. There is no significant sex predilection, but it is probably more common in later life. The diagnosis is base only on histologic examination that represents, in the best of cases, the only way to differentiate the COC from other pathologies. Such lesions include: odontogenic cyst, residual cyst, globulomaxillary cyst, lateral periodontal cyst, ameloblastoma (multicystic and unicystic), odontogenic mixoma, and still with calcifying odontogenic epithelial tumor (Pindborg tumor) and adenomatoid odontogenic tumor. The authors report a case of COC, starting point for a review of the clinical, radiographic and histomorphologic features of the lesion.