This case report further expands the known phenotypes of BEST1 by describing a mother and daughter who were found to have genetic diagnosis of BEST1, c.1014_1015delGAinsCT;p.(Trp338_Asn339delinsCysTyr) and presented with fundus findings of extensive pisciform fleck and central macular atrophy phenocopying Stargardt disease. Here, BEST1 is linked to Stargardt disease.