KCNV2-associated retinopathy, also known as cone dystrophy with supernormal rod responses (CDSRR), is a type of autosomal recessive cone-rod dystrophy caused by pathogenic variants in KCNV2 (OMIM *607604), and was first described by Gouras et al. in 1983 (1). The gene discussed is KCNV2; the disease is autosomal recessive cone rod dystrophy.