We describe the case of a 42-year-old woman with giant cavernous hemangioma and Kasabach-Merritt syndrome. The patient presented with consumption coagulopathy due to intravascular, intratumoral coagulation as revealed by low platelet levels, fibrinogenopenia and an increase in fibrinolysis with high levels of fibrinogen degradation products. She was scheduled to receive an orthotopic liver transplant because of three factors: respiratory distress caused by compression of the diaphragm by the giant tumor; the risk of bleeding caused by spontaneous rupture or trauma; and the presence of Kasabach-Merritt syndrome due to consumption coagulopathy. Before surgery fibrinogen deficit was corrected with 4 units of cryoprecipitates and low platelet level was treated with 10 units of platelets. Coagulopathy during surgery was corrected with fresh plasma (17 units), cryoprecipitates (6 U), aprotinin (1 x 10(6) U/kg) and antithrombin 3 (2000 U). Blood loss was compensated for with 9 units of packed red blood cells. This report describes the procedures used for anesthesia, for prevention of accidental bleeding during surgery, hemodynamic control and preoperative coagulation testing.