Stargardt disease (STGD1), also known as fundus flavimaculatus or ABCA4-retinopathy, is a progressive disorder of the retina caused by bi-allelic variants in the gene that encodes ATP-binding cassette subfamily A member 4 (ABCA4), localized on chromosome 1p22.1. Here, ABCA4 is linked to severe early-childhood-onset retinal dystrophy.