Mutations in the human Crumbs homolog 1 (CRB1, MIN #604210) gene lead to severe congenital and early-onset retinal dystrophies (EORD) with a wide range of clinical manifestations including Leber congenital amaurosis (LCA, MIN #613835), early-onset Retinitis Pigmentosa (EORP) with or without preserved para-arteriolar retinal pigment epithelium (PPRPE; MIN #600105), Coats-like exudative vasculopathy, and juvenile cone-rod dystrophies [1-5]. The gene discussed is CRB1; the disease is severe early-childhood-onset retinal dystrophy.