Background <p>Congenital dysfibrinogenemia is a rare autosomal dominant disorder involving abnormal fibrinogen function, leading to variable risks of bleeding and thrombosis. Its management during pregnancy is particularly challenging due to the potential for complications such as miscarriage, stillbirth, placental abruption, and fetal growth restriction. The absence of reliable predictive laboratory markers further complicate individualized risk assessment and clinical decision-making.</p> Case presentation <p>A 23-year-old primigravida was diagnosed with congenital dysfibrinogenemia after low fibrinogen levels were detected early in her pregnancy. Family history revealed similar fibrinogen abnormalities in her father and brother. Amniocentesis for fetal genetic testing was performed after a multidisciplinary team review that evaluated the bleeding risks, with no complications during or immediately following the procedure. Genetic testing confirmed congenital dysfibrinogenemia in both the mother and fetus. Throughout the pregnancy, the patient was closely monitored, with no signs of bleeding, thrombosis, or other complications. At 39.3 weeks, following a failed attempt at vaginal delivery, she underwent a cesarean section after fibrinogen replacement therapy. The surgery was uneventful, and there were no significant bleeding or thrombotic events postoperatively.</p> Conclusions <p>This case emphasizes the critical role of a multidisciplinary team approach in managing congenital dysfibrinogenemia during pregnancy, amniocentesis, and perinatal care. It demonstrates the effectiveness of thorough disease assessment and fibrinogen replacement therapy in preventing bleeding, thereby ensuring good outcomes for both the mother and fetus.</p>