FOXC1 lies in the 6p25 forkhead cluster (FOXC1/FOXF2/FOXQ1) and mutations in this gene cause a spectrum of autosomal dominant anterior eye segment defects, including Axenfeld-Rieger syndrome type 3 (ARS; MIM#602482), causing an increased risk for glaucoma and varying degrees of iris or extra-ocular abnormalities [16]. This evidence concerns the gene FOXC1 and Axenfeld-Rieger syndrome type 3.