Disease-causing variants in KCNV2 are present in a substantial fraction (2%-5%) of different cohorts with the broad clinical diagnoses of a cone dysfunction/dystrophy, suggesting that Cone dystrophy with supernormal rod response may be underdiagnosed.6, 7, 8 In total, we identified 114 cases described in the literature, in 22 studies and case reports.9 The gene discussed is KCNV2; the disease is cone dystrophy.