A 44 year-old woman with a family history of abdominal aortic aneurysm (AAA) underwent resection of an AAA. Histological examination revealed a lack of elastic fibers of the media and a diminished content of smooth muscle cells. A congenital hypoplasia of the aortic media was postulated as the etiology of this AAA. This condition may represent an aortic variety of medial defect observed in intracranial berry aneurysms, and be associated with a hereditary predisposition to AAAs.

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