Among the monogenic diabetes associated with extra-pancreatic features, there are diseases with an autosomal dominant (HNF1B-MODY and GATA6 syndrome) or autosomal recessive type of inheritance (DIDMOAD, Walcott–Rallison syndrome, and Bardle–Biedl syndrome), insulin resistance type A, and diabetes inherited through the maternal line (mitochondrial diabetes) [2,4,5,6,7,8,9,10,11]. The gene discussed is GATA6; the disease is diabetes mellitus.