Biallelic autosomal recessive variants in PROM1 have been documented to cause retinitis pigmentosa (RP), CRD, or panretinal dystrophy of cone-rod phenotype with early macular involvement6–11, while autosomal dominant variant mainly result in Stargardt-like macular dystrophy (STGD4) or CRD with a later onset and a slower decline in visual function10–17. Here, PROM1 is linked to cone-rod dystrophy.