Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by café au lait macules, neurofibromas, and iris hamartomas. Internal carotid artery aneurysms rarely affect patients with NF-1 but may be associated with rupture or neurologic complications. We report a 42-year-old female patient with NF-1 and a bilobed right internal carotid artery aneurysm extending to C2. The aneurysm was excised with high exposure of the carotid artery and reconstruction was performed using saphenous vein graft. We review the literature relevant to internal carotid aneurysms associated with NF-1.