Ehlers-Danlos syndrome (EDS) type IV results in a high incidence of vascular lesions. The extreme fragility of arteries is associated with multiple aneurysm formation, spontaneous rupture, and dissection. Surgical management of patients with this disorder is hazardous and often unrewarding. In this report we describe the difficulties encountered in the management of three patients with EDS-related vascular lesions. Three patients presented with pain and exhibited characteristic features of EDS. Diagnostic modalities included computerized tomography, transesophageal echocardiography, and magnetic resonance. Aortography was performed only in specific situations. One patient with pericardial and mediastinal hemorrhage was stabilized and treated conservatively, with a good outcome. An adolescent with a ruptured aortic pseudoaneurysm died at surgery. The third patient underwent successful surgical correction of multiple aortic and renal aneurysms. In view of the increased risk of fatal vascular complications, surgeons should identify patients with EDS before performing invasive procedures. Arteriography should be used only when necessary. Although operative mortality remains at a high level due to the tendency of vessels to tear with even minimal manipulation, mortality from hemorrhage without surgical intervention is even greater. The key to favorable outcomes lies in identification of the syndrome preoperatively, surgical intervention only in life- or limb-threatening situations, and appropriate modification of surgical technique.