Neurofibromatosis type 1 (NF-1), or von Recklinhausen's disease, is an autosomal dominant disorder that primarily affects the skin, the central and peripheral nervous systems, and the skeleton. Vasculopathy, such as, aneurysm or stenosis of large vessels is a less common manifestation of NF-1, but spontaneous aortic dissection in NF-1 is very rare. We report a case of acute aortic dissection with incidental aneurysm of the right femoral artery in NF-1.