A 13-year-old girl with a 1-year history of elevated serum alanine transferase was hospitalized because of liver failure. Low prothrombin time (12%) prevented needle liver biopsy. Because of a high titer of antismooth-muscle antibodies (1:500), a tentative diagnosis of "autoimmune" chronic active hepatitis was made and immunosuppressive therapy was started. Despite the severity of the liver disease, of her poor general condition, and of spontaneous bacterial peritonitis, she dramatically responded to treatment, prothrombin time returning to normal within 5 months. Diagnosis of chronic active hepatitis was later confirmed by liver biopsy. This report indicates that immuno-suppressive therapy can be life saving in children with severe chronic active hepatitis even when major signs of liver failure are present.