Factor X deficiency is one of the rarest inherited coagulation disorders. It is an autosomal recessive inherited disease. In its homozygous form factor X deficiency has an estimated prevalence of 1: 500,000. However in its heterozygous form it has an estimated frequency of 1: 500 to 1: 2000. Pregnancy in women with congenital factor X deficiency has been associated with adverse foetal outcomes. We report a case of pregnancy in a woman with factor X deficiency. She was treated early during labour with prophylactic replacement of prothrombin complex concentrates (Kaskadil). An initial infusion of 40 UI.kg-1 of factor X was followed by 20 UI.kg-1 every 24 hours during three days. During labour and peripartum maternal coagulation was screened. She delivered a healthy baby at 33 weeks of gestation. No episode of abnormal bleeding was observed. Therefore in this case, prophylactic therapy using prothrombin complex concentrates during labour and delivery did prevent severe haemorrhages.