The patient, a 40-year-old woman had a bleeding diathesis at age nine. Von Willebrand's disease was diagnosed. After age 16, she had multiple surgical procedures and blood transfusions. At age 34 intermittent thrombocytopenia was noted. She required hospitalizations which included exploratory surgical procedures, transfusions, trials of ethinyl estradiol and norethindrone acetate, prednisone, splenectomy, and massive transfusions. Her factor VIII level ranged between 8 and 39 per cent. No gastrointestinal lesion has been identified. The patient's mother had von Willebrand's disease, intermittent thrombocytopenia, and also chronic gastrointestinal bleeding. No underlying disease has been found to account for either the thrombocytopenia or the severe gastrointestinal bleeding.The long survival of both the patient and her mother is attributed to blood transfusions and, for our patient, the arteriovenous shunt which was inserted.