The odontoameloblastoma (OA), also known as ameloblastic odontoma, is a rare neoplasm of jaws which includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behaviour. The exact incidence is difficult to determine. Since 1944, only 24 cases have been reported in English literature which fulfill both histological and clinical features of this lesion. The Authors report a case report of an odontoameloblastoma in a 15-year-old caucasian man treated with a surgical excision. The five years follow-up shows no evidence of recurrence confirming the validity of a conservative surgery with enucleation of OA, followed by periodical clinical and radiographical controls.