The most common juvenile retinal dystrophy, STGD is a phenotype characterized by variable macular atrophy with white–yellow deep retinal lesions in the posterior pole and extending to the mid-peripheral retina.[137] These so-called “pisciform” flecks are variably hyper-AF due to the buildup of RPE LF.[138] It is classically associated with autosomal recessive (AR) mutations in the ABCA4 gene although autosomal dominant (AD) ELOVL4 and PRPH2/RDS have been reported.[139,140]. Here, PRPH2 is linked to severe early-childhood-onset retinal dystrophy.