Neonatal diabetes mellitus (NDM) is a rare cause of hyperglycemia in the neonatal period. We encountered a patient with neonatal diabetes mellitus and exocrine pancreatic insufficiency secondary to a homozygous insulin promoter factor-1 (IPF1) mutation. We sought to review the available literature regarding current etiologies and therapeutic options available to patients with NDM. Identification of genetic mutations and further investigation of neonatal diabetes mellitus has allowed for improvement in patient care and a better understanding of the etiology of neonatal diabetes mellitus. Given the rarity of the condition, the neonatologist, pediatrician and family practitioner must be aware of these changes in order to organize and facilitate appropriate care.