While recent reports are detailing the ophthalmological features of visually impaired BBSOAS children (Zou et al., 2020; Jurkute et al., 2021), mouse models offer again the unique opportunity to further understand the role of Nr2f1 in vision, from the developmental and the functional points of view. Here, NR2F1 is linked to Bosch-Boonstra-Schaaf optic atrophy syndrome.