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Aim Methods Results Conclusion Recent evidence suggests that acute liver failure (ALF) in some patients may reflect a dysregulated immune response, and that corticosteroids improve survival of the native liver in ALF patients with high serum alanine aminotransferase levels, which are an indication of liver inflammation. However, it is unclear whether steroids are effective for pediatric acute liver failure (PALF). The aim of this retrospective case–control study is to examine whether steroid therapy for PALF accompanied by immune activation improves the survival of native liver and to identify factors that predict responses to steroid treatment.Of 38 patients with PALF treated at Kyoto University Hospital from February 2006 to August 2022, 19 receiving steroids who met the specific criteria for identifying the pathophysiology of immune activity in the liver (the “Steroid group”), and seven steroid‐free patients who also met the criteria (“Nonsteroid group”) were enrolled. Patients in the “Steroid group” were categorized as “responders” or “nonresponders” according to treatment outcome. Clinical and histological data were analyzed.Survival of the native liver in the Steroid group was significantly higher than that in the Nonsteroid group (68% vs. 0%, respectively; p = 0.0052). Nonresponders were significantly younger, with higher Model for End‐stage Liver Disease and pediatric end‐stage liver disease scores, higher prothrombin time – international normalized ratio, and higher serum ferritin levels than responders. Massive hepatic necrosis was more common in nonresponders.Steroid therapy is effective for PALF patients with liver inflammation; however, liver transplantation should be prioritized for young children with ALF accompanied by severe coagulopathy or massive hepatic necrosis. [ABSTRACT FROM AUTHOR]