Activation of the coagulation process is frequently encountered in patients with venous vascular malformations and results in the local formation of clots and the consumption of components of the coagulation process (platelets, clotting factors). This activation accounts for multiple biological abnormalities such as the elevation of the D-dimers, reduction of fibrinogen and platelets count and less frequently a local intravascular coagulation (LIC). This process seems to be responsible for the local painful symptoms. In case of LIC, bleeding complications may be observed. Therapeutically, antithrombotic treatment with low-molecular weight heparin can improve the local coagulopathy with beneficial effects on blood tests disturbances, pain and bleeding tendency.