Diffuse venous malformations can be associated with a consumptive coagulopathy characterized by a reduction of fibrinogen level, platelet count and elevated D-dimer level. We report a case of a patient with extensive venous malformations, hemorrhagic symptoms and biological signs of intravascular coagulopathy. She was initially treated effectively with low-molecular weight heparin (LMWH) (enoxaparin 1 mg/kg, bid) and switched to low-dose dabigatran etexilate (110 mg bid) for more than 2 years. Both treatments showed a similar clinical efficacy with the absence of bleeding or thrombotic complications. Compared with LMWH, dabigatran etexilate provided a similar correction of the fibrinogen level and platelet count but was less effective to reduce the D-dimer level. Although dabigatran etexilate can be safely used to control the consumptive coagulopathy secondary to venous malformation and provides a practical alternative to LMWH, its efficacy in vivo at a low dose to reduce the D-dimer level was lower than that of LMWH.