Vascular abnormalities are recognized but rare manifestations of Neurofibromatosis type 1.

Stenoses, aneurysms and pseudoaneurysm are the most common vascular abnormalities.

External carotid aneurysm has not been reported before in the litterature.

The most common clinical presentation of aneurysms is their spontaneous rupture.

Carotid aneurysm rupture can cause a life-threatening upper airway obstruction.

Carotid aneurysm in NF-1 can be treated by surgical or endovascular approach or combination of the both.