Papilliferous Keratoameloblastoma (PKA) is a rare entity and not much is known about its clinicodemographic features or biological nature. Our review aimed to provide clarity with respect to the characterization of demographic, clinical, radiological, and histopathological features of PKA. Case reports of PKA were identified by means of a systematic search across multiple databases. The search yielded a total of 10 cases, half of which were of Indian origin. All the cases invariably occurred in the mandibular posterior region and involved the right side, except for one case which primarily involved the left side of the mandible. PKA should be considered as a variant of conventional ameloblastoma but towards the more aggressive end of the spectrum. It tends to occur in older individuals (fifth decade or older), with a marked propensity to occur in the right mandibular posterior region. Surgical resection with diligent follow-up is warranted in the treatment of PKA.
Keywords: Odontogenic tumors; Ameloblastoma; Keratin; Odontogenic keratocyst