The Bernard-Soulier syndrome (BSS) is a rare disorder of platelet function which is characterized by a prolonged bleeding time, giant platelets, and thrombocytopenia. Treatment of hemorrhage in patients with BSS usually requires platelet transfusion, but alloimmunization frequently occurs after repeated transfusions. In such patients prevention of bleeding may be difficult. In the literature there are a few cases of patients with BSS suffering from recurrent bleedings and refractory to platelet transfusions who were treated successfully with factor VIIa. Here, we describe another BSS patient refractory to platelet transfusions who had presented with severe gastrointestinal bleeding and was treated successfully with recombinant factor VIIa.