No patients with NIPHS have been identified as having mutations in the Kir6.2 or SUR1 genes as occurs in certain children with a syndrome of familial hyperinsulinemic hypoglycaemia; however, the two groups do share similar histologic findings within the pancreas, namely, nesidioblastosis - a histologic diagnosis consisting of widespread β-cell hyperplasia along with diffuse proliferation and hypertrophy of islet cells from pancreatic ducts [1,2,5-7]. Here, KCNJ11 is linked to familial hyperinsulinism.