The iliac vein compression syndrome is important cause for lower venous hypertension, and frequently manifests as lower extremity varicose veins and deep venous thrombosis. With the development of diagnostic technology and rising awareness, an increasing number of patients with iliac vein compression are being identified. There are presently some controversies concerning the treatment of iliac vein compression syndrome complicated with lower extremity varicose veins or deep venous thrombosis. In this paper, the authors present the recent viewpoints as well as diagnostic and treatment advances on iliac vein compression syndrome.