History. It was not until 1962 that calcifying odontogenic cyst was described as a separate entity among the odontogenic tumors (Gorlin et al. 1962). The lesion of the jaw mentioned by Rywkind (1932), who compared it to cholesteatoma of the ear, was later identified as a calcifying odontogenic cyst (Gorlin et al. 1962; Altini and Farman 1975). The atypical case of an ameloblastoma described in 1946 by Thoma and Goldman as a “strange variant” of an ameloblastoma was subsequently classified as a calcifying odontogenic cyst. Since 1962 more than 70 cases have been reported in the literature.