Ameloblastoma is a non-encapsulated, slow growing tumor with high recurrence rate. Orbital Involvement by this neoplasm is a very rare entity. In this study, we present the clinical and paraclinical features of a case and a systematic literature review on this situation.

An electronic search was made on major medical sources. Data of the cases in the literature in addition to our own case were extracted; summarized; and statistically analyzed.

We bring the 5 years history of a 50-years-old man with orbital/skull base invasion of Plexiform maxillary ameloblastoma. A total of 35 other cases from 19 relevant studies were also reviewed. Review topics included: Epidemiology; Clinical presentation; Pathologic features; Differential Diagnosis; Imaging Treatment; and, Prognosis.

Maxillary ameloblastoma is a locally aggressive neoplasm and physicians must be alert about the biologic behavior of this tumor to detect any invasion to critical structures like orbit and cranium. We advocate meticulous patient follow-up with regular clinical examinations and para-clinical work-up for timely detection of any invasion or recurrence. Best must be done to avoid extensions by complete removal of maxillary ameloblastoma aggressively. Orbital ameloblastoma causes significant morbidity and mortality.