A young woman suffering from sideropenic anemia, bleeding disorders related to von Willebrand's disease and pulmonary vascular telangiectasias presented at our observation. Laboratory findings, genetic and hemostatic studies revealed platelet aggregation defect while in her father and grandfather lung angiodysplastic lesions without platelet aggregation defect. The association between bleeding disorders and pulmonary angiodysplasia has rarely presented in literature and deficiency of von Willebrand factor might increase the risk of bleeding in patients with coexisting angiodysplastic disease.