The ameloblastoma (AB) is a true neoplasm of enamel organ type tissue which does not undergo diff erentiation to the point of enamel formation. AB is locally invasive and recurs despite adequate surgical removal. It is of varied origin, although the stimulus initiating the process is unknown. The study of molecular and genetic alterations associated with the development and progression of the AB will help to predict the course of the tumor and lead to the development of new therapeutic concepts for their management. An attempt has been made at compiling about molecular pathogenesis of AB.