Ameloblastoma is uncommon odontogenic tumor and the granular cell variant is one of the most rare and controversial type of ameloblastoma described. The granular cell ameloblastoma occurs predominantly in the posterior region of the mandible. The tumor demonstrates a marked propensity to recur following conservative therapy with eventual distant metastasis. We herein report a case, review the literature and discuss the importance of the subtyping the granular cell ameloblastoma.