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Infective endaortitis with a splenic abscess is an extremely rare and fatal complication of coarctation of the aorta. We herein describe a 19-year-old female patient with a 2-month history of intermittent fever with progressive abdominal and leg pain. Aortic computed tomography angiography showed a rapidly progressive poststenotic saccular aneurysm immediately distal to the coarctation. Enhanced abdominal computed tomography showed a severe splenic abscess. During the operation, the infected spleen was resected first. We subsequently removed all of the diseased aorta and performed in situ aortic reconstruction with an artificial tube graft and without extracorporeal circulation. The patient had an uneventful postoperative clinical course and recovered very well during the 4-year follow-up. In summary, our case report details the successful surgical treatment of coarctation of the aorta with infective endaortitis and a splenic abscess and thoroughly discusses the indications and key notes for surgery without extracorporeal circulation in this complex disease.