Ameloblastic fibro-odontoma (AFO) is a rare, benign, and mixed odontogenic tumor that consists of both ectodermal and mesenchymal elements. AFO is more prevalent in young children and adolescents than in adults and is usually found in the molar area associated with a failure of tooth eruption. The purpose of this report is to discuss the differential diagnosis and treatment of a three-year-old girl diagnosed with an AFO around a primary canine. The manifestations of the lesion resembled localized periodontal disease caused by an enamel pearl. Excision and curettage were done and the separated dental hard tissue was confirmed from the enamel structure of the primary canine. In addition to the hard tissue, pulpy and soft tissues were removed together and were histologically examined, confirming the diagnosis of AFO.