Hepatorenal syndrome (HRS) is a functional and reversible impairment of renal function in patients with severe cirrhosis. Major pathophysiological elements include liver dysfunction, a circulatory derangement with central hypovolaemia and neurohumoral activation of potent vasoactive systems leading to a pronounced renal vasoconstriction. The prognosis of patients with HRS is poor but recent research has spread new enthusiasm for treatment. Efforts at treatment should seek to improve liver function, to ameliorate arterial hypotension and central hypovolaemia, and to reduce renal vasoconstriction. Therefore a combined approach should be applied with reduction of portal pressure with e.g. ss-adrenergic blockers and transjugular intrahepatic portosystemic shunt (TIPS), with amelioration of arterial hypotension and central hypovolaemia with vasoconstrictors such as terlipressin and plasma expanders. New experimental treatments with endothelin- and adenosine antagonists and long-acting vasoconstrictors may have a future role in the management of HRS.