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Nonalcoholic steatohepatitis occurs commonly in adults with obesity or diabetes mellitus. There are only a few reports of this condition in children.Prospective consecutive clinical series.Between December 1985 and April 1995, 36 children (21 boys, 15 girls) were diagnosed with nonalcoholic steatohepatitis at the Hospital for Sick Children, Toronto. The median age at diagnosis was 12 years (range, 4–16 years). Most patients were referred because of elevated serum aminotransferases or abnormal hepatic sonogram. Thirty patients (83%) were obese. Two patients had diabetes mellitus at diagnosis, and it developed later in two. Fifteen patients had palpable hepatomegaly, and one of these had splenomegaly. None had physical signs of chronic liver disease. Thirteen of 36 patients had acanthosis nigricans. Serum aminotransferases were elevated in all but one patient. Tests for Wilson disease and chronic hepatitis B and C were negative. Serum lipid profiles were abnormal in 18 patients: 7 had hypercholesterolemia, and 11 had hypertriglyceridemia. Twenty-four of 31 examined had abnormal liver sonograms suggestive of fatty infiltration. Twenty-four patients underwent percutaneous liver biopsy: all showed large-droplet fat. Inflammation was present in 88% and fibrosis-cirrhosis in 75%. One 10-year-old patient had established cirrhosis at diagnosis.Nonalcoholic steatohepatitis occurs in children, is clinically diverse, and may not be benign.