The causes frequently ascribed to coarctation of the abdominal aorta include atherosclerosis, trauma, inflammatory aortitis, and extrinsic compression. We present a case of coarctation of the abdominal aorta with stenosis of the left renal artery due to fibrodysplasia in a 35-year-old woman, hospitalized for treatment of intermittent claudication and hypertension. This coarctation was resected and end-to-end anastomosis with reimplantation of the left renal artery was performed. Results of an anatomopathologic examination of the resected aortic specimen and the trunk of the left renal artery revealed typical lesions of fibromuscular dysplasia. The specific features of lesions in this case were compared with those from the literature.