ObjectiveTo explore the etiology and biochemical markers of acute liver failure (ALF) in children.Methods The cause and the biochemical markers of ALF in children who were treated in December 2014 to January 2011 were ana-lyzed retrospectively.ResultsA total of 67 children were enrolled, including 31 females and 36 males. According to the cause of the disease, the children were divided into non-genetic metabolic group, genetic metabolic group, and cryptogenic group. In the non-genetic metabolic group (29 cases, 43.28%) there were 12 cases of drug-induced ALF, 5 cases of Reye syndrome, 3 cases of hemophagocytic syndrome, 3 cases of herpes simplex virus infection, 2 cases of autoimmune hepatitis, one of case mushroom poisoning one case of hepatitis A virus infection, one case of cytomegalovirus infection and one case of sepsis respectively. In the genetic metabolic group (14 cases, 20.90%) there were 6 cases of Wilson’s disease, 2 case of glycogen storage disease, 2 of cas-es progressive familial intrahepatic cholestasis, 2 cases of neonatal intrahepatic cholestasis caused by citrin deifciency, one case of very long-chain acyl coenzyme A dehydrogenase deifciency and one case of primary carnitine deifciency. In the cryptogenic group there were 24 cases (35.82%). The serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), albumin, blood glucose level and AST/ALT were statistically signiifcantly different in genetic metabolic group from in non-genetic me-tabolism disease group and cryptogenic group, (P<0.05). The genetic metabolic group had the lowest levels of serum ALT, AST, albumin and glucose while the genetic metabolic group had the highest ratio of AST/ALT.ConclusionsThe etiology of ALF in children are complex. Genetic metabolic disease should be considered when the child with ALF has no signiifcantly elevated ALT, extremely high ratio of AST/ALT, combined with hypoproteinemia and hypoglycemia.