Congenital hyperinsulinism is an inherited condition affecting the pancreatic β cells, leading to overproduction of insulin and persistent low blood sugar levels. The most severe type of hyperinsulinism arises from mutations in ABCC8 or KCNJ11 genes, encoding the subunits of two ATP-sensitive potassium channels found in β cells. Diazoxide is the only FDA-approved treatment for hyperinsulinism, but patients with these mutations are unresponsive to this drug and need a pancreatectomy. Therefore, novel therapies for severe hyperinsulinism are urgently needed.