More than 50% of PNDM cases are due to activating mutations in KCNJ11 and ABCC8, while majority of mutations genes associated with PNDM are de novo in origin (Gloyn et al., 2004; Babenko et al., 2006; Edghill et al., 2010). The gene discussed is KCNJ11; the disease is permanent neonatal diabetes mellitus.