Hepatorenal syndrome (HRS) is defined by progressive changes in the splanchnic and systemic circulation of cirrhosis patients. It is usually secondary to triggering events, inducing a complex multiorgan dysfunction syndrome, also including renal failure with a reduction in urinary output. The progression rate of the renal dysfunction discriminates between HRS type 1, where the worsening is more rapid, i.e. 1-2 weeks, and type 2, where instead it has a slower progression. The sympathetic nervous system, the renin-angiotensin system, antidiuretic hormone, cytokines and endothelial factors are all involved at the same time and are mutually interactive. Sodium and water retention, with ascites, edema and dilutional hyponatremia on the one hand and glomerular filtration inhibition due to intrarenal vasoconstriction on the other, are the main clinical manifestations. In the past, HRS resolution was dependent on the possibility of a liver transplant, which is usually followed by the restoration of normal renal function. Nowadays, pharmacological therapy based on the use of vasoconstrictors adds new steps to HRS treatment. Terlipressin, an analogue of vasopressin, in combination with albumin may lead to renal recovery in 40-60% of patients. Moreover, in the bridge phase to transplantation, the new systems for the extracoporeal depuration of the renal failure solutes and protein-bound solutes typical of liver failure could increase the short-term patient survival. HRS alone should not be considered an indication for combined kidney-liver transplant. In patients with advanced cirrhosis, the prevention of complications possibly triggering HRS is based on prophylactic antibiotics in order to avoid contamination of the ascitic fluid and albumin to increase the plasma volume.