Mauriac syndrome, characterized by the combination of glycogenic hepatopathy and stunted growth in the setting of poorly controlled T1DM, has become increasingly rare with the availability of multiple daily insulin injections, insulin pumps, and closed-loop systems in developed countries but remains underdiagnosed in patients with long-standing poorly controlled T1DM [1,2,8,17,20]. The gene discussed is INS; the disease is Mauriac syndrome.