Newfoundland rod-cone dystrophy (NFRCD) is characterized by night blindness that is present in infancy, along with a progressive loss of peripheral, central and color vision beginning in childhood. The end result is severe vision loss by the second to fourth decade of life. The optic nerve is either normal or minimally pale until a late stage of disease. The macula is normal or may exhibit a “beaten-bronze” atrophy. Young patients also exhibit a perimacular ring of white stippling and a scallop-bordered lacunar atrophy of the mid-peripheral retinal pigment epithelium, which is similar in appearance to early gyrate atrophy or choroideremia.