We describe the ultrasound-Doppler and angio-CT findings of the internal carotid string-of-beads sign in a patient with fibromuscular dysplasia (FMD). FMD is a disease of unknown etiology typically affecting the medium and larger arteries of young and middle-aged women. The most commonly affected arteries are the renal arteries followed by the internal carotid arteries. The lumbar, mesenteric, celiac, hepatic and iliac arteries are less commonly affected. Patients typically present with renovascular hypertension in case of renal artery involvement, Transient ischemic attack or stroke can be one of the presenting symptoms in carotid artery involvement.