Key Points: Bernard–Soulier Syndrome

BBS is a rare inherited platelet adhesion disorder characterized by thrombocytopenia and an increased risk of postpartum hemorrhage. Platelet transfusion remains an important hemostatic strategy, while recombinant activated factor VII may be considered in selected cases, particularly in the setting of platelet refractoriness or alloimmunization risk. Tranexamic acid is commonly used as adjunctive or prophylactic therapy during delivery and the postpartum period. Delivery should occur at a specialized center with multidisciplinary planning and access to transfusion support. Regional anesthesia is generally contraindicated due to the risk of spinal or epidural hematoma.