This article reports an unusual case of Axenfeld-Rieger Syndrome (ARS) associated with severe maxillofacial and skeletal anomalies. A 55-year-old man with ARS revealed interesting and unexpected radiological findings. Severe maxillofacial and skeletal anomalies, in addition to the well-recognized extraocular findings, that occurs in patients with ARS is reported and our case highlights this possible rare association between ARS and maxillofacial and skeletal anomalies.
Keywords: Axenfeld-rieger syndrome; corectopia; dextrocardia; maxillary aplasia; pseudopolycoria.