Introduction: Ischemic necrosis of the liver (INL) is caused by reduction of the hepatic blood supply, and is defined by acute, reversible elevation of serum transaminases, after exclusion of other causes of hepatocellular injury. INL usually occurs during hypotensive events, leading to hepatocyte hypoxia. This report of a baby with advanced biliary atresia (BA) cirrhosis illustrates the clinical and biological findings, liver hemodynamics and histological results of focal INL and discusses its pathogenesis. Case Report: A 8 months old boy with BA, with previous successful portoenterostomy (post-operative bilirubin 15μmol/l), was admitted with jaundice, fever, stool discoloration, and increasing ascites. Ascending cholangitis was suspected and treated, followed by clinical improvement. Eight days later he was febrile again. ASAT and ALAT rose to 9018 and 2346UI/l, synthetic liver function deteriorated. Infectious work-up was negative. Liver-ultrasound showed reversed flow in the hypoplastic portal vein and a negative arterial diastolic flow (hepatic artery resistance-index >1.0). This episode occurred while feeding the child with hyper-caloric hyper-osmolar milk, without presenting hemorrhage or hypotension. The patient recovered within 5 days under supportive treatment. A second similar event occurred, and semi-urgent living related (mother) liver transplantation (LT) was performed 2 weeks thereafter. Histology of the explanted liver confirmed the diagnosis of advanced biliary cirrhosis, with metachronous foci of INL. Post-operative course was uneventful and mother and child are doing well one year after surgery. Conclusion: This case illustrates the correlation between hemodynamic alterations of hepatic blood supply and the occurrence of foci of INL in BA cirrhosis, leading to rapid deterioration of liver function and need for urgent LT. Since splanchnic hemodynamic alterations may be triggered by hyper-osmolar hyper-caloric enteral feeding, such preparations should be used cautiously prior to LT, with close monitoring of hepatic blood flows.