Angiodysplasia of the gastrointestinal tract consists of ectasia of the submucosal vessels of the bowels and may lead to acute or chronic bleeding. The presence of a coagulopathy will increase the frequency and the severity of gastrointestinal bleeding, whether spontaneously or medically. In literature, few cases of Bernard-Soulier syndrome associated with gastrointestinal angiodysplasia have been reported. We hereby present a female patient known to have Bernard-Soulier syndrome presenting with persistent bleeding due to jejunal angiodysplasia, which, to our knowledge, is the eighth reported case in the medical literature. The patient responded to tranexamic acid (antifibrinolytic agent) with gradual reduction in required transfusions until cessation of bleeding. (c) 2013 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.