Calcifying odontogenic cyst (COC) or calcifying cystic odontogenic tumor was first introduced in 1962 by Gorlin et al., as a possible oral counterpart of calcifying epitheliomas of Malherbe in skin. This lesion is a rare odontogenic lesion with variable clinico-histological characteristics. Three different histologic subtypes has been reported for COC. In this study we presented a female patient diagnosed with ameloblastomatous COC a very rare variant of this lesion and challenges regarding microscopic diagnosis and treatment of it is discussed.