Germline pathogenic variants in <i>FOXC1</i> are associated with anterior segment dysgenesis and Axenfeld-Rieger syndrome (ARS, #602482), an autosomal dominant condition with ophthalmologic anterior segment abnormalities, high risk for glaucoma and extraocular findings including distinctive facial features, as well as dental, skeletal, audiologic, and cardiac anomalies. The gene discussed is FOXC1; the disease is Axenfeld-Rieger syndrome.