A 72-year-old man was referred to our hospital for examination of anemia. Upper gastrointestinal endoscopy revealed a giant ulcerative lesion in the lesser curvature of the stomach and the pathological diagnosis of the biopsy specimen was poorly differentiated adenocarcinoma. Laboratory data showed prolonged PT-INR and factor VII coagulating activity reduced to 1.7%. We diagnosed advanced gastric cancer with congenital factor VII deficiency and performed total gastrectomy, cholecystectomy and splenectomy with administration of recombinant activated factor VIIa (rFVIIa) just before incision. Additional use of rFVIIa was performed after operation, but was not on the following day. Two days after surgery, bloody drainage was observed and PT-INR prolonged to 5.26. rFVIIa was used for more two days and bleeding became less severe. Fresh frozen plasma (FFP) was also given for three days from the third day after surgery. Thereafter he had no more complications and was discharged. Although the patient underwent an operation with congenital factor VII deficiency, especially with high decrease of factor VII coagulating activity, rFVIIa should be administered considering the risk of postoperative bleeding.