We describe our surgical experience of localized thoracoabdominal aneurysm in a 60-year-old woman with hypertension and hyperlipidemia. She was admitted for severe nausea associated with uremia. The initial CT scan revealed bilateral hydronephrosis, retroperitoneal fibrosis, inflammatory abdominal aneurysm, and localized thoracoabdominal aneurysm. To resolve the bilateral urinary tract obstruction, bilateral ureteral stents were inserted. After the renal function improved, the thoracoabdominal aneurysm was removed and replaced with an 18-mm woven-Dacron graft under partial cardiopulmonary bypass. The inflammation and fibrosis along the abdominal aorta did not extend to the thoracoabdominal aneurysm. Following the case presentation, we discussed the pathophysiologic aspects of this patient.