A patient with congenital factor VII deficiency (baseline activity, 7%) underwent surgery for hepatocellular carcinoma. No literature reference was found concerning the management of this coagulation defect in patients requiring liver surgery. We report one such case, with special reference to the peri-operative management of factor VII replacement therapy and the pharmacokinetic behavior of factor VII. As a result of accidental suprahepatic vein lesion, a bleeding episode occurred. This was associated with an increase in factor VII clearance and volume of distribution values from 1.30 to 3.85 l/h, and from 6.51 to 13.2 l, respectively. After surgery, the patient continued to receive the concentrate every 8 h during the first nine post-operative days. No post-operative bleeding or thrombotic event was observed. The patient was discharged in good condition.