GATA6 syndrome is characterized by a combination of neonatal diabetes mellitus (up to 72.7% of cases [6]), exocrine pancreatic insufficiency (up to 67.3% of cases), and congenital developmental anomalies including the heart (up to 89.9% of cases) and the gastrointestinal tract [6,12,13,14]. The gene discussed is GATA6; the disease is exocrine pancreatic insufficiency.