Hepatorenal syndrome (HRS) is a common complication of advanced cirrhosis, characterized by renal failure and major abnormalities in the systemic circulatory function. Renal failure is caused by intense vasoconstriction of the renal circulation. The syndrome is probably the final consequence of an extreme underfilling of the arterial circulation, secondary to vasodilatation in the splanchninc vascular bed and a decrease in cardiac output due to central hypovolemia. The diagnosis of HRS is based on the exclusion of other causes of renal failure. The survival of patients with HRS is very short, particularly when there is rapidly progressive renal failure (type-1 HRS). Liver transplantation is the best therapeutic option but its applicability is low. During the past few years effective treatment for HRS, such as vasoconstrictor drugs (vasopressin analogues, ∝-adrenergic agonists) associated with intravenous albumin infusion and transjugular intrahepatic portosystemic shunts (TIPS), have been introduced. They improve circulatory function, normalize serum creatinine, and may improve survival. Sequential treatment with vasoconstrictors plus albumin and TIPS is an attractive therapeutic possibility. Plasma volume expansion with albumin at infection diagnosis in patients with spontaneous bacterial peritonitis and the administration of pentoxiphilline in patients with severe alcoholic hepatitis significantly reduce the development of type-1 HRS.