Hepatorenal syndrome (HRS) is a form of acute kidney injury (AKI) that occurs in children with significant acute or chronic liver disease. This condition is characterized by lower renal function with decreased renal blood flow that is unresponsive to fluid expansion and is associated with increased morbidity and mortality. In decompensated liver disease, mortality is reported up to 50% in children with HRS-AKI stage 2–3. Splanchnic vasodilation and systemic inflammation activation are central in its development. Early recognition and supportive management, including fluid expansion with albumin, may initially reverse some AKI. Patients unresponsive to this treatment and fulfilling HRS-AKI criteria must receive vasoconstrictor agents and albumin infusions to treat this renal dysfunction. Failure to respond is associated with worst outcome and increased mortality. Unless the underlying liver disease is transient or significantly improves, liver transplantation may be required in HRS-AKI.