Congenital hyperinsulinism (CHI), a group of disorders with clinical, genetic, and morphological heterogeneity, is one of the important causes of persistent recurrent hypoglycemia in infancy and childhood. Accordingly, congenital hyperinsulinemia can be classified into the following seven genetic types: ATP-sensitive potassium channel hyperinsulinemia (KATP-CHI), glutamate dehydrogenase-type hyperinsulinemia. (GDH-CHI), short-chain 3-hydroxyamino-CoA dehydrogenase hyperinsulinemia (SCHAD-HI), glucokinase-type congenital hyperinsulinemia (GK-HI), hepatocyte nuclear factor 4 alpha hyperinsulinemia (HNF4A-HI), mitochondrial uncoupling protein 2 hyperinsulinemia (UCP2-HI)) and exercise hypoglycemia/monocarboxylic acid Molecular genetic studies have confirmed that more than 50% of congenital hyperinsulinemia is caused by mutations in the above eight genes, while about 33%-50% of CHI patients are not found to carry mutations in the above genes, suggesting that further genetic studies of CHI patients are extremely necessary.