KCNV2 encodes Kv8.2, an electrically silent voltage-gated potassium channel subunit expressed in photoreceptor inner segments 1. This protein functions as a potassium channel modulator, shifting the activation threshold of K+ channels to more negative values, thereby regulating channel activity in photoreceptors. KCNV2 mutations cause cone dystrophy with supernormal rod response (CDSRR), an autosomal recessive cone-rod dystrophy characterized by pathognomonic electroretinogram findings 2. Patients with KCNV2-associated retinopathy typically present before age 12 with severely decreased visual acuity affecting all patients, reduced color vision (78.6%), photophobia (53.5%), nyctalopia (43.6%), and nystagmus (38.6%) 3. KCNV2-deficient photoreceptors show upregulation of genes associated with apoptosis, oxidative stress, and hypoxia pathways 1, suggesting the primary dysfunction involves photoreceptor degeneration rather than cardiac complications. The disease demonstrates slow peripheral retinal progression with largely stable electrophysiologic findings across 6 decades 3. Currently, no treatment is available, but the identification of a potential intervention window until age 40 4 combined with recent development of gene therapy vectors 1 suggests therapeutic prospects are emerging.