We report on a case of a distal aortic arch aneurysm with severe shaggy aorta treated by two-stage hybrid surgery without complications. The patient was a 67-year-old man. The thoracic aortic aneurysm was identified on computed tomography imaging by his treating physician during routine follow-up for lung cancer. The patient was referred to our hospital for further investigation and treatment. The aneurysm had a maximum diameter of 68 mm with severe shaggy aorta. Based on these findings, the risk of cerebral infarction and spinal cord ischemia was considered very high. The patient underwent total arch replacement with elephant trunk, using a brain isolation technique for this initial surgery. The postoperative course was uneventful and thoracic endovascular aortic repair（TEVAR) was performed 26 days after the initial surgery. The patient was discharged on post-operative day 38, without complications.