Congenital hypodysfibrinogenemia is characterized by the synthesis of an abnormal fibrinogen molecule that does not function properly when converting fibrinogen into fibrin. The functional defects include: abnormal fibrinopeptide release, defects in fibrin polymerization, abnormal fibrin stabilization, and resistance to fibrin lysis. The most common hypodysfibrinogenemias are those causing polymerization defects. In most cases, congenital hypodysfibrinogenemia is inherited as an autosomal dominant trait with high levels of penetrance, but some patients show an autosomal recessive inheritance. Clinically, patient with hypodysfibrinogenemia will have one of the following phenotypes: no hemorrhagic symptoms, mild bleeding following trauma, thrombosis, or both thrombotic and hemorrhagic symptom manifestations (1). Approximately, 43% of all individuals with congenital hypodysfibrinogenemia are asymptomatic, while about 20% have bleeding symptoms and 17% experience thrombotic manifestations. About 20% of patients have a combination of bleeding and thrombosis (2). Women with congenital hypodysfibrinogenemia are at greater risk of obstetric complications. We report on a 31-year-old woman kept under our observation after having delivered a healthy baby successfully during the 40th gestational week. Thirty days after the end of pregnancy, a cerebral venous thrombosis (occlusion of left transversal sinus) was diagnosed, because of a sudden dyplopia. She was examined for inherited and acquired thrombophilia. Antithrombin III, protein C, protein S, activated protein C resistance, homocysteinemia, lupus-like inhibitor, and anti-phospholipid antibodies were normal. The research for the mutation of Factor V Leiden and the mutation G20210A of prothrombin was negative. The coagulation parameters were Partial Thromboplastin Time (PTT) ratio: 1.15, Prothrombin Time (PT) INR: 1.18, thrombin time: 120 seconds, ratio 4.78, reptilase time: 53 seconds, ratio 2.10, fibrinogen level: 69 mg/dl, fibrinogen antigen level: 90 mg/dl. Then, a diagnosis of hypodysfibrinogenemia was followed, and the patient began oral anticoagulant therapy with warfarin. Then, the patient asked to interrupt warfarin to start a new pregnancy. Therefore, therapy with Enoxaparin 6000 IU antifactor Xa once in day was started (100 IU/kg of body weight). This therapy and the dosage were considered appropriate as prophylaxis of recurrent vein thrombosis. The antifactor Xa activity was in therapeutic range (0.5–1.0 IU) in scheduled controls. At 18th week of pregnancy, the patient was admitted in hospital for sudden abdominal pain and vaginal bleeding. A moderate abruptio placenta was seen by echotomography. The following day, because of a serious vaginal hemorrhage and severe anaemia (Hb: 6.9 g/dl) and increase in the retroplacentar ematoma, the heparin therapy was stopped; a replacement therapy with fibrinogen concentrate and packed red cells was started. On 19th week of pregnancy, the patient delivered a dead fetus of 200 g body weight. No specific therapy is indicated for patients with congenital hypodysfibrinogenemia who are asymptomatic. Hypodysfibrinogenemic patients who have thrombotic episodes require anticoagulation with low molecular weight heparin followed by oral anticoagulants (3). Recurrent thrombotic episodes require prophylactic anticoagulation. Replacement treatment with fresh-frozen plasma, with cryoprecipitate, a rich source of fibrinogen, or with fibrinogen concentrates solvent-detergent treated, is indicated for episodes of active bleeding, preoperatively, and during pregnancy (4, 5). Prophylactic therapy is only recommended during pregnancy, and women with recurrent spontaneous abortions and hypodysfibrinogenemia can be treated with fibrinogen replacement therapy throughout the course of pregnancy. Our case is uncommon, because there were hemorrhagic and thrombotic symptoms in the same patient during the pregnancy period. The first pregnancy was complicated in the immediate postpartum by or because of a thrombotic episode in an unusual site. In the second pregnancy, she had a still birth under dramatic circumstances (placental abruption and serious bleeding). We believe in the case of another pregnancy, the patient must be treated both with heparin and with fibrinogen concentrates to avoid the risk of abortion (and bleeding) and thrombotic complications.