Axenfeld-Rieger syndrome type 1 (Monarch Disease Ontology entry MONDO_0008386) is a rare autosomal dominant syndrome linked to mutations in the PITX2 gene. It is characterized by abnormalities in the anterior chamber of the eye and underdevelopment of the teeth. Also known as: Axenfeld-Rieger syndrome caused by mutation in PITX2, PITX2 Axenfeld-Rieger syndrome, RIEG1, Rieger syndrome type 1.