To date, more than 150 disease-associated variants in CRB1 (OMIM #604210) have been described, associated with a range of inherited retinal disease (IRD) phenotypes including Leber congenital amaurosis (LCA), early as well as adult-onset retinitis pigmentosa (RP)—with and without a Coats-like vasculopathy, and more recently macular dystrophy and foveal schisis [1–11]. Here, CRB1 is linked to Leber congenital amaurosis.