We describe a previously healthy 48-year-old man who presented with clinica
l characteristics suggestive of internal carotid artery dissection, confirm
ed by magnetic resonance imaging. He developed a massive infarction of the
left cerebral hemisphere and died after 3 days of transtentorial herniation
. Postmortem examination identified a dissection of the thoracic aorta caus
ed by Erdheim-Gsell cystic medionecrosis, with the characteristic degenerat
ion of the elastic fibers of the lamina media. The dissection showed an unu
sually large extension not only distally into both iliac arteries, but also
proximally into both carotid arteries. To our knowledge, such an extensive
dissection has not been described previously. Underlying vessel wall disor
ders of the aorta, such as Erdheim Gsell cystic medionecrosis, should be co
nsidered in young patients with spontaneous arterial dissection.