Four patients had fibromuscular dysplasia of the extremities involving the brachial, deep femoral, popliteal, anterior and posterior tibial and peroneal arteries. One of them demonstrated a string-of-beads appearance in the superficial veins of the arm and this was pathologically confirmed as fibromuscular dysplasia. We believe this is the first report of this disease affecting the peripheral arteries and the superficial veins of the extremities. The sympathetic system seems to be related to the development of fibromuscular dysplasia.