Calcifying odontogenic cyst is considered as a rare lesion and accounts for 1% of jaw cysts. It represents a heterogeneous group of lesions which exhibit a variety of clinicopathologic and behavioral features. It has been categorized as cyst and neoplasm. Calcifying odontogenic cyst is an uncommon developmental odontogenic cyst first described by Gorlin in 1962. It is considered as an extremely rare cyst and accounts for only 1% of jaw cysts reported. Because of its diverse histopathology, here has always been confusion about its nature as a cyst, neoplasm, or hamartoma. WHO classification 2005 has reclassified the lesion as calcifying cystic odontogenic tumor (CCOT) in this report, we present a rare case of calcifying odontogenic cyst –an extremely rare histologic variant in a 29 year–old female in the maxillary anterior region. The lesion was surgically removed. After enucleation no recurrence has been recorded in the ensuing 2 years. Radiological features: CCOT are generally a unilocular lesion, in 5–13% multilocular. Three patterns of radiopacity seen: 1st salt and pepper pattern of flecks, 2nd fluffy cloud like pattern, and 3rd crescent–shaped pattern on one side of the radiolucency in a “new moon”–like configuration. Histopathological features: Stroma showed thick bundles of collagen fibers with spindle fibroblasts, Juxta–epithelial hyalinization, Daughter cysts with ghost cells, Dysplastic dentin in and around some odotogenic islands. With the above histopathologic features, a diagnosis of ameloblastomatous CCOT was made. Results: The patient has been under regular follow up and has not exhibited any signs of recurrence after 2 year of follow up.