Liver cirrhosis is associated with number of hematological complications and coagulation disturbances. In view of various haemostatic abnormalities it is surprising that many patients do not bleed spontaneously. Severe coagulopathy of liver disease is more frequently seen in acute liver failure, but still remains important complication of liver cirrhosis and chronic liver failure. Decreased production of blood coagulation factors by the liver plays a key role in altered haemostasis in liver diseases. Altered fragile balance of blood coagulation proteins and infection are associated with both worsening coagulopathy and bleeding risk. Additional haemostatic abnormalities in patients with severe liver diseases are thrombocytopenia, chronic disseminated intravascular coagulation, accelerated fibrinolysis, hypofibrinogenemia and dysfibrinogenemia. In this review we discuss a complicated issue of multiple coagulopathies in patients with advanced liver dysfunction.