Ameloblastomatous calcifying odontogenic cyst (COC) is a very rare histopathological variant of COC, which is an odontogenic cyst of the jaw. The term "calcifying odontogenic cyst" is not covered in the World Health Organisation (WHO) Classification of Tumors 2005 and was re-named as calcifying cystic odontogenic tumour (CCOT). There are only a few reports containing details on CCOT being associated with ameloblastoma. This variant has been classified as ameloblastomatous CCOT (type 3) as per the WHO 2005 classification. In this article, we reported an exceptional case of ameloblastomatous CCOT in a 15-year-old boy involving the mandibular anterior region, which is a rare combination for age and site of the lesion, along with an impacted tooth which is again an uncommon association.