Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder characterized by cutaneous pigmentations, neurofibromas, Lisch nodules and neuroectodermal tumors. Supra-aortic vessel aneurysms may affect patients with NF-1 and can be associated with rupture, ischemic complications and compression symptoms. We describe a 48-year-old woman with NF-1 and an extracranial 3×5 cm right internal carotid artery aneurysm. After balloon test occlusion the patient was treated with parent artery sacrifice which led to significant shrinkage on follow-up MR and reduction of compression symptoms. The literature concerning internal carotid artery aneurysms associated with NF-1 is reviewed evaluating the possible therapeutic options.