Wolcott–Rallison syndrome (WRS, OMIM: 226980) is caused by an autosomal eukaryotic translation initiation factor 2-a kinase 3 (EIF2AK3) deficiency, associated with permanent diabetes and skeletal dysplasia in newborn period or early infancy, hepatic dysfunction, and growth retardation, which was first reported in 1972 by Wolcott and Rallison (1–3). Here, EIF2AK3 is linked to diabetes mellitus.