Eight cases of chronic hepatitis with marked hypergammaglobulinemia occurring in pediatric patients are reported. Arthralgia, abdominal pain, skin rashes, and bleeding tendencies were present in these patients, but none had a positive lupus cell preparation. Administration of adrenocortical steroid drugs was followed by clinical improvement in four cases and no significant change in one. Despite the administration of adrenocortical steroid drugs, three patients died. At autopsy, postnecrotic cirrhosis was found in each of these three cases. The etiology of the syndrome of chronic hepatitis with marked hypergammaglobulinemia has not been established. Cases occurring in children do not appear to differ significantly from cases in adults. There is some evidence that autoimmunization against hepatic tissue is important in pathogenesis, but this hypothesis has not been definitely established.