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Background and Aims Case report of a 29-year-old woman, 38 weeks gestation, that came to our pre-anesthetic consultation with personal history of congenital FVII deficiency with history of gingivorrhagia and, as a remarkable antecedent, major bleeding after mammoplasty that required 24 h admission to the ICU, despite having administered rFVIIa (Novoseven) before the operation. Aims: Individualised analysis of this particular case and establishment of recommendations for the delivery agreed by a board of experts.Methods A serch of the available scientific evidence in databases (PubMed, UpToDate....).Results Both in the choice of anesthetic technique and type of delivery, the personal history of postoperative hemorrhage despite Novoseven prophylaxis weighed heavily. Therefore, the apparently safest option was chosen: cesarean delivery under general anesthesia. The delivery was completely normal and without complications. Bleeding was controlled without requiring hematite concentrate transfusion or extra doses of Novoseven.Conclusions Until larger studies and clear guidelines are available, the management of labor in women with FVII deficiency should be addressed on a case-by-case basis in multidisciplinary meetings involving obstetrics, anesthesiology and hematologist with expertise in hemostasis. FVII level alone does not predict bleeding risk; management discussions should take into account the patient‘s bleeding history, third trimester PT, multiple gestation, and mode of delivery. In healthy women, during pregnancy, concentrations of many coagulation factors increase and may have a protective effect against bleeding. The risk of thrombotic complications associated with pregnancy and cesarean section is independent of FVII levels.