The subnormal EOG in this patient can be associated with reduction of the scotopic ERG and is milder than the reported abolished EOG light rise in a patient with a similar retinal phenotype in dominant RPE dystrophy due to the p.Asp477Gly allele in RPE65. 13RGR and RPE65 are expressed in the RPE and these alleles likely result in cellular toxicity.5,13,18 The subnormal macular function, as shown on PERG in one asymptomatic carrier, suggested that this retinopathy can involve the macula. Here, RPE65 is linked to retinal disorder.