The BPES phenotype observed in children with NR2F2 mutations is strikingly similar to that associated with dominant mutations in FOXL2. These pathogenic variants cause BPES frequently together with 46,XX premature ovarian insufficiency but testis development has not been reported. BPES is thought to originate following developmental defects in peri-ocular mesenchymal cells, which are required to form the levator smooth muscle, tarsus, and Meibomian glands. Coup-tf2 is known to play crucial roles in the formation of the morphology of the murine eye, and given the similarities in the phenotypes, it would be of interest to determine whether FOXL2 and COUP-TF2 are acting in the same developmental pathway.24