Axenfeld-Rieger syndrome refers to a group of developmental diseases with developmental defects in both eyes, with or without systemic developmental abnormalities. Its characteristics are: ① developmental defects in both eyes; ② may be accompanied by systemic developmental abnormalities; ③ secondary glaucoma; ④ autosomal dominant inheritance, often with a family history, and there are also reports of sporadic cases; ⑤ the disease occurs equally in both sexes. In 1920, Axenfeld reported a patient with a raised white line near the corneal margin, and a tissue strip extending from the peripheral part of the iris to this white line. Axenfeld called it the posterior embryotoxon of the cornea. In the mid-1930s, Rieger reported a group of patients with the same anterior segment abnormalities, but there were other changes in the iris, such as ectopic pupils, iris atrophy, and hole formation. Some of these patients also had developmental abnormalities other than the eyes, especially dysplasia of the teeth and facial bones.