Hepatorenal syndrome (HRS) is a dreaded complication of advanced cirrhosis, with dismal prognosis if left untreated. Recent understanding of the pathophysiology underlying HRS development has led to many new treatments. Vasoconstrictors (eg, midodrine, terlipressin, or norepinephrine) together with albumin can improve the effective arterial blood volume with increased renal perfusion pressure. This combination has been proven successful in reversing HRS in approximately two thirds of patients. Transjugular intrahepatic portosystemic shunt (TIPS), which reduces portal pressure and decreases the sympathetic drive associated with portal hypertension, is effective in improving renal function. The combination of a TIPS and vasoconstrictor therapy has been shown to normalize renal function in a small number of patients. Liver transplantation corrects many of the pathophysiological abnormalities associated with HRS, and is the best treatment option. Patient outcome is improved further if the renal function is normalized prior to liver transplantation.