The simultaneous occurrence of ameloblastomas with odontogenic cysts or other non-odontogenic lesions have already been described as combined lesions. However, we are unaware of any report in the English literature of simultaneous occurrence of ameloblastoma and orthokeratinized odontogenic cyst (OOC) occurring as completely distinct lesions. This report shows a case of synchronous ameloblastoma and OOC, located on posterior regions of the mandible, but in distinct sides.