The adenomatoid odontogenic tumor is a relatively uncommon lesion which mainly affects females in their second decade of life, exhibiting predilection for the anterior region of the maxilla. The lesion is usually associated with the crown of an enclosed tooth, most commonly the maxillary canine. In this paper we present a case of adenomatoid odontogenic tumor associated with a dentigerous cyst affecting the left maxillary region in a 13-year-old female. The authors also discuss clinical, radiographic, histopathologic and therapeutic features of the case.