The central function of the liver as place of the formation of the most plasmatic coagulation factors in expressed by coagulation disturbances in severe liver diseases. Apart for intoxications, vitamin-K-deficiency and relatively rare congenital dysproteinoses the synthesis of the coagulation factors is apparently a relatively stable and rapidly recoverable function of the hepatocyte. On the other hand, however, the pathogenetic dominance of the accelerated balance in the sense of a disseminated intravasal coagulation and a secondarily increased fibrinolysis is emphasized for the severe disturbances of haemostasis in hepatopathies. In all kinds of shock, in tumours, inflammations, sepsis and intoxications as well as in portal hypertension of any reason the activation of the coagulation system up to the consumption coagulopathy develops. In the liver transplantation in addition to the operation shock deterioratingly comes the intensive blood contact with the often heavily damaged graft cells, the effectors of the immune system (rejection), the temporary disturbance of the clearance function of the liver RES and the basic disease of the recipient which in most cases disposes to the disseminated intravasal coagulation. The reduction of thrombocytes and coagulation factors extensively transgresses the changes which are to be expected by a weakness of the synthesis during and immediately after the anhepatic phase. For the therapy the demand of rapid removal of the coagulation-activating moments (minimum times of ischaemia, careful anastomoses) and of an adequate substitution with simultaneous prevention of the disseminated intravasal coagulation by heparinisation.