The hepatorenal syndrome (HRS) is a unique form of acute renal failure with entirely normal renal histology in advanced liver disease. Its diagnosis is made by exclusion of all causes of renal failure and by all the five major criteria as set by the International Ascites Club. The presence of hepatomegaly, poor nutritional status, and oesophageal varices at endoscopy are associated with a high risk of HRS. The liver tests, the Child-Pugh score, are of no value in prediction of its occurrence. Contraction of the effective blood volume, which may lead to renal hypoperfusion with preferential renal cortical ischaemia, is proposed pathogenesis of the condition. Because understanding of the pathogenesis of HRS is incomplete, therapy is supportive only. Optimal fluid management is vital as there is almost invariably a reduction in effective arterial blood volume. Dopamine, frusemide and haemofiltration may be helpful in management of fluid overload but do not affect renal function. TIPS has been used successfully in small series of patients. The vasopressin analog also has been used with early excellent response. The treatment of HRS has been discouraging and the only proven cure for HRS is liver transplantation at this point of time.