Reye syndrome is characterized by an encephalopathy and fatty infiltration of the liver and other organs. Reye syndrome is usually associated with influenza B or varicella. Frequently these patients have been treated with aspirin-containing compounds. Other disorders may mimic the phenotypes of Reye syndrome, and these conditions include various intoxications and some inborn problems of metabolism. This is to report our experience in a case of Reye like syndrome combined with the aspirin medication after Kawasaki disease. We present this case with a brief review of related literatures.