KCNV2-associated retinopathy (cone dystrophy with supernormal rod responses; OMIM #610356) is an unusual autosomal recessive retinal disorder and a leading cause of cone-rod dystrophy.1, 2, 3 The KCNV2-retinopathy Study Group is a multicenter international retrospective investigation of clinically and molecularly confirmed patients with the disease. This evidence concerns the gene KCNV2 and Rod-cone dystrophy.