A case of fibromuscular dysplasia (FMD) with involvement of the aorta is described in a 44-year-old woman. So far only 3 cases of aortal FMD have been mentioned, all in female patients. Our observation is characterized by a solitary stenosis of the lower abdominal aorta and it resembles histologically the “intimal fibroplasia” type of renal FMD. The review of the literature about FMD in general reveals that mostly the renal arteries are affected, followed by cervical artery FMD with only rare occurrence in other vessels. Though the etiology is basically unknown several factors have been made responsible, namely hereditary disposition, congenital rubella infection, female hormones, paucity of the vasa vasorum, and stretch-traction-stress. The disease has been classified into several histological subgroups and also according to the roent-genologic form which typically shows a “string of beads” appearance. There is a definitive female sex preponderance of 5:1, which is the same for all affected vessels; whereas in respect to age there is a marked distinction in so far that renal FMD patients on average are 11 years younger than patients with the cervicocephalic form of the disease.