Hypoglycemia is a common metabolic disorder in children,and its incidence is about 10％.Among them,the incidence of hereditary related diseases is about 1/30 000-10/30 000.More and more genetic diseases leading to hypoglycemia have been discovered in recent years.Children with the disease may have mild nervous system damage at the earliest stage,and liver encephalopathy,respiratory failure,confusion or even death in severe cases.Therefore,more and more attention has been paid to the study of hypoglycemia related genetic diseases,but the diagnosis and differential diagnosis of hypoglycemia has not yet formed an unified consensus.Now,the diagnosis and treatment of this kind of related diseases were summarized in order to provide data for clinical practice.