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Coagulation alterations might represent a problem in obstetric anesthesia considering that they may contraindicate neuraxial techniques and worsen a case of uterine atony with more severe bleeding if they are not correctly recognized and treated. We report the case of a parturient diagnosed with severe factor XI deficiency during the delivery progress. In this case, non-steroidal anti-inflammatory drugs and neuraxial techniques were avoided and intravenous patient-controlled analgesia with boluses of remifentanil was used for pain management. Treatment with tranexamic acid and fresh frozen plasma was initiated and the absence of urgent factor XI availability was notified. Due to no progression of labor, cesarean section was required and a general anesthesia was performed. During the procedure, uterine atony occurred. Uterine massage and several uterotonic drugs were needed to control it. The patient remained stable and the delivery was accomplished without further incidents. The objective of this report is to present the pain, coagulation and bleeding management of a patient with hemophilia C in our obstetric department and to alert for the need of multidisciplinary work to successfully approach this type of patient.