Both neonates had hyperglycemia in the first month of life of more than 250 mg/dL making subcutaneous insulin an ideal treatment for such cases of pancreatic agenesis causing PNDM [13]. Lastly, in 2018, Evliyaoğlu et al. reported three cases of neonatal diabetes, with two having isolated pancreatic agenesis due to PTF1A mutation, while the third neonate developing epilepsy and developmental delay alongside pancreatic agenesis with KCNJ11 mutation [21]. Here, KCNJ11 is linked to pancreatic agenesis.