KCNV2-associated retinopathy (cone dystrophy with supernormal rod responses; OMIM #610356) is a rare form of autosomal recessive (AR) inherited retinal disease (IRD) with a pathognomonic electroretinogram (ERG).1, 2 The KCNV2 retinopathy study group is the largest multicentre retrospective investigation of affected individuals. Here, KCNV2 is linked to cone dystrophy.