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To review the incidence, etiologies, pathophysiology, and treatment of acute liver failure (ALF) in children. Emphasis will be placed on the initial management of the multiple organ system involvement of ALF.MEDLINE search from 1970 to March 2005 was performed. Search headings were as follows: acute liver failure, fulminant liver failure, pediatric liver failure, hepatic encephalopathy, and liver transplantation. Studies written in English were selected. Pediatric studies were emphasized. Adult studies were referenced if there were no pediatric studies available in regard to a specific aspect of liver failure.Pediatric acute liver failure is a rare but life-threatening disease. The common etiologies differ for given age groups. Management includes treating specific causes and supporting multiple organ system failure. Commonly associated disorders that require initial recognition and treatment include energy production deficiencies (hypoglycemia), coagulation abnormalities, immune system dysfunctions, encephalopathy, and cerebral edema. Criteria used to determine the need for liver transplant are reviewed as well as the difficulties associated with predicting which patients will meet these criteria and how rapidly liver transplant will become the only option. Finally, experimental procedures that may provide additional time for the liver to recover are briefly reported.