A 26-year-old lady with Loeys Dietz syndrome had undergone 5 consecutive operations from 2007 to 2014. Finally, her total aorta was replaced by vascular grafts and stent grafts. The patient was admitted to our hospital with headache and dizziness in 2016. Computed tomography showed a giant pseudoaneurysm at the aortic arch with extravasation from the vascular graft. The proximal flares of thoracic stent grafts caused perforation of thoracic vascular grafts. Cerebral perfusion was not sufficient, because this aneurysm compressed the innominate, left carotid and left subclavian arteries. Decreasing of cerebral perfusion caused headache and dizziness. We performed total arch replacement. We started extracorporeal circulation before median sternotomy but the aneurysm ruptured during median sternotomy. We controlled the bleeding by manual compression and immediately started selective cerebral perfusion and induced cardiac arrest. After opening the distal portion, we cut down the proximal flares of thoracic stent graft and inserted an elephant trunk into the stent graft. We sutured between the new vascular graft and the stent graft and covered the suture line with another short vascular graft. As a result we could protect the vascular graft from the stent graft. Computed tomography demonstrated that the pseudoaneurysm and extravasation disappeared. Cerebral perfusion increased and the patient's symptoms improved. Postoperatively the patient was discharged from our hospital without any major complications at POD 11.