Ehlers-Danlos syndrome (EDS) is a rare inherited disorder of the connective tissue that is characterized by hyperextensible skin, hypermobile joints and abnormalities of the cardiovascular system. A 15-year-old girl with Ehlers-Danlos syndrome underwent thoracolumbar surgery for deformity correction. After surgery, an abdominal aortic rupture occurred, and she complained of abdominal distension had an abdominal circumference of 80 cm. Abdominal computed tomography revealed a pseudoaneurysm and a large hematoma at the retroperitoneum. She died of a massive hemorrhage during subsequent abdominal aortic surgery.