Hepato-renal syndrome (HRS) is one of the most detrimental conditions in patients with end stage liver failure. Historically, HRS was considered a terminal disease associated with cirrhosis and was termed "liver-death syndrome". Furthermore, despite the improved understanding of pathophysiology and the reversibility of renal dysfunction in HRS, mortality remains extremely high especially for type 1 HRS. This review summarizes the recent advances in the pathophysiology, diagnosis and management of HRS and also provides an evolving area of research in the pathophysiologic mechanisms of HRS, which may open the door for new therapeutic approaches.