Abstract: Factor Ⅺ deficiency is a hereditary blood coagulation disorders. Puerpera with factor Ⅺ deficiency is exceedingly rare. A case of a 28-year-old woman who was found to have factor Ⅺ deficiency coagulopathy at the end of 33 weeks of gestation was presented here, and was admitted to Peking University Third Hospital at the end of 38 weeks of gestation. The doctors from several departments conducted cooperative management, and prepared with supplement of the coagulant factor and fresh frozen plasma before operation. The woman had general anesthesia, the baby was delivered successfully and the mother’s condition was kept stable. The amount of bleeding of puerpera was not much perioperation. Yet the management of cesarean section with factor Ⅺ deficiency at home and abroad is still lack of standard guideline by now.

Key words: Factor Ⅺ deficiency, Cesarean section, Anesthesia