The Calcifying Odontogenic Cyst (COC) represents a heterogeneous group of lesions that exhibits a variety of clinicopathologic and behavioural features. Therefore a proper categorization of the cases is needed for better understanding of each variant. Ameloblastoma is one of the well-known odontogenic tumours that could be associated with Calcifying Odontogenic Cyst. Very few cases of Ameloblastomatous calcifying odontogenic cyst have been reported in the literature. In this report we present a case of ameloblastomatous transformation of calcifying odndontogenic cyst.