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Secondary sclerosing cholangitis in critically ill patients (SSC-CIP) is a rare disease in adult patients undergoing intensive medical treatment and is almost undescribed in childhood. In patients without pre-existing liver disease, a severe underlying disease with critical hypoperfusion of the vascular plexus and subsequent ischemic injury of the intrahepatic bile ducts (e.g. due to shock or sepsis) leads to rapidly progressive biliary destruction with only few causal therapeutic options. The diagnostic gold standard is endoscopic retrograde cholangiography (ERC) with the possibility of an intervention, especially since noninvasive diagnostics, e.g. sonography, magnetic resonance cholangiography (MRC) have a poor sensitivity in very young patients. In high-grade peripheral bile duct loss and biliary liver cirrhosis, liver transplantation is the only curative treatment approach. The SSC-CIP is an entity that is likely to be diagnosed more frequently in the future and is a relevant complication in critically ill children after survival of the underlying disease. This article describes the clinical courses in three children with SSC-IP who rapidly developed biliary cirrhosis and were successfully treated with a liver transplantation.