Although orodental anomalies have been reported to occur in patients with oculoauriculovertebral spectrum, no previous publications in the English literature have been located that describe an ameloblastic fibroma in a patient with this condition. This report presents a 15-month-old female monozygotic twin with concomitant oculoauriculovertebral spectrum and a congenital ameloblastic fibroma of the mandible. A theory linking the pathogenesis of the two conditions is also raised.