Buerger's disease is also known as thromboangiitis obliterans, and it is a nonatherosclerotic, segmental, inflammatory, vasoocclusive disease that affects the small- and medium-sized arteries and veins of the upper and lower extremities. It is classified in the miscellaneous category of vasculitis. Although Buerger's disease has a worldwide distribution, it is now considered to be more prevalent in the Middle East, Near East and Far East than in North America and Western Europe. Even though there are some differences between the diagnostic criteria, the commonalities are an earlier onset of distal ischemic symptoms; a smoking history; infra-popliteal or brachial arterial occlusive lesions; either upper extremity involvement or phlebitis migrans; the absence of atherosclerotic risk factors or other distinct arterial occlusive diseases. Although smoking, genetic predisposition, hypercoagulability, endothelial dysfunction and autoimmune mechanisms have been proposed as the etiologic factors of Buerger's disease, there is no single etiologic mechanism present in all the patients with the disease. However, it seems that the condition is strongly associated with heavy smoking and the progression of the disease is also closely linked to continue smoking. The marked cellular proliferation and inflammatory infiltrate in the thrombus is considered as a pathologic hallmark of the disease. The clinical presentations of Buerger's disease range from intermittent claudication to ischemic ulcer. Small finger or toe tip ulcers, superficial thrombophlebitis, Raynaud's phenomenon, sensory findings and an abnormal Allen's test are relatively unique signs and symptoms. It seems that the degree of dependent rubor or migrating thrombophlebitis is an easily recognizable sign that represents aggravation or alleviation of the disease activity. The typical "corkscrew collaterals" are well visualized at the late stage of Buerger's disease. Although various therapies are now available, the only cornerstone therapy is complete cessation of cigarette smoking or the use of tobacco in any form. Because the natural course of life and limb is favorable in many patients with Buerger's disease, any harmful interventional treatments have to be avoided.