Congenital factor VII deficiency is a rare disease. We performed gastrectomy safely in a gastric cancer case with congenital factor VII deficiency after administering recombinant activated factor VII (rFVIIa) preoperatively. The patient, who was a 58-year-old man, underwent examination to treat hepatitis B and was given a diagnosis of gastric cancer with congenital factor VII deficiency. Prothrombin time was prolonged to 18.0 seconds (control 12.7 seconds), and factor VII activity was reduced to 10%. Upper gastrointestinal endoscopy showed gastric cancer on the anterior wall of the gastric angle. We performed distal gastrectomy after intravenous injection of rFVIIa 1.2 mg (18 μg/kg) preoperatively. After distal gastrectomy, 1.2 mg of rFVIIa was injected intravenously for a total of 13 times by postoperative day 4, after measuring factor VII activity. Few cases of congenital factor VII deficiency have been reported in surgery, and distal gastrectomy was performed safely by measuring factor VII activity and administering rFVIIa.