According to statistics, China’s annual birth of about 20 million newborns, of which 400,000 to 500,000 may suffer from inherited metabolic diseases, can be diagnosed genetic metabolic liver disease has more than 600 kinds, including carbohydrate metabolism, amino acid metabolism, fatty acid metabolism, organic acid metabolism, mitochondrial hepatopathies, lysosomal disorders, peroxisomal diseases, metal metabolism disorders, and other diseases such as α1-antitrypsin deficiency and so on nine categories. trypsin deficiency and other nine major categories. The prevalence of individual diseases is not high, but the overall prevalence should not be ignored. We analyzed the changes in the disease spectrum of non-viral liver diseases in children hospitalized from 2001.1 to 2001.12, and the results showed that the most common non-viral liver diseases in children were liver metabolism-related diseases, which accounted for 46.2% (325/703) of the total number of hospitalizations. Because of the late start of clinical and research work on inherited metabolic liver diseases in China, the lack of social and medical workers’ awareness of these diseases, and the limited detection technology and diagnostic means, there is a lack of large-scale epidemiological data on inherited metabolic liver diseases in children in the mainland of China at the present time. Inherited metabolic liver disease is mainly caused by metabolites or cholestasis resulting in damage to liver cells, but the liver’s response to the damage is very limited in form, so the clinical manifestations of metabolic liver disease are not specific, such as fatigue, loss of appetite, nausea, vomiting, bloating, diarrhea, or even coma, etc.; the physical signs are mainly jaundice and hepatosplenomegaly, or the presence of developmental delays. Therefore, it must be differentiated from other acquired liver damage, including infectious liver damage such as viral, bacterial, mycoplasma, toxoplasma, drug-induced liver damage, non-alcoholic steatohepatitis, autoimmune liver disease, toxic liver damage, and tumors. Metabolic liver disease can also overlap with other acquired liver damage, especially in our country viral hepatitis and very common hepatophilic virus (CMV,EBV) hepatitis incidence is higher, the chance of overlap is higher, making the clinical manifestations more mixed, bringing more difficulties to the diagnosis, and more prone to unnecessary omission of the diagnosis, misdiagnosis and mistreatment. According to statistics, more than 30% of patients with genetic metabolic diseases have to go through 5-10 doctors; 48.3% of patients are misdiagnosed as other diseases; and the time of misdiagnosis is up to 5 years or longer. In conclusion, the clinical manifestations of genetic metabolic diseases are complex and non-specific, so they need to rely on special experimental techniques for diagnosis.