Fibromuscular dysplasia (FMD) of the extracranial internal carotid arteries is a relatively rare angiographical finding which has been reported as an incidental finding during angiography, as well as in association with a variety of different clinical conditions. A case is reported of transient attacks of amaurosis fugax and hemiparesis. Angiography in this patient revealed bilateral FMD of the internal carotids as well as a small ulcerative plaque involving the right internal carotid artery at the region of carotid bifurcation. Surgical treatment directed toward the ulcerative plaque and not the FMD of the carotid arteries has rendered the patient asymptomatic. Lack of symptoms, despite the persistence of FMD, suggests that FMD is of doubtful pathogenic significance.A small, surgically correctable ulcerative plaque should not be overlooked in the presence of FMD of the internal carotid arteries. The significance of the rare finding of FMD of the internal carotid arteries is not clear.