【Abstract】 Hereditary coagulation factor V deficiency (HFVD) is a rare blood clotting disorder. The main manifestations of the patients are mucosal hemorrhage and post-traumatic hemorrhage. The Coagulation function tests indicate significant prolongation of prothrombin time and activated partial thromboplastin time, and a remarkable decrease in coagulation factor V activity. Patients with this type of disease have a very high risk of surgical bleeding, and the anesthesia management is complicated. We reported a 39-year-old female patient with hereditary coagulation factor V deficiency and the preoperative coagulation factor V activity of 1.8%, who underwent anesthesia management of laparoscopic hysterectomy and bilateral salpingectomy under general anesthesia due to the abnormal uterine bleeding. Meanwhile, the relevant literatures on perioperative management of this disease were reviewed in this article.