Vascular involvement in thoracic outlet syndrome is beside neurological symptoms one of the possible manifestations of this disease, typically affecting young, otherwise healthy individuals. The most frequent vascular manifestations of the thoracic outlet syndrome include symptoms related to the venous hypertension with possible outfall to the deep vein thrombosis. Arterial involvement is mostly revealed by the presence of ischemic symptoms. Several key points for the correct diagnosis should be stressed out. They include specific events in patient history, especially previous effort or trauma of the affected upper extremity, typically unilateral. Arterial manifestations are characterized by the absence of ischemic symptoms in other localisations. Deep vein thrombosis occurs in the absence of other distinct precipitating factor and/or hypercoagulable state. Some provocative manoeuvres may help in correct diagnosis assessment, especially in the connection with duplex ultrasound examination. Endovascular procedures both in venous and arterial thoracic outlet syndrome became rather a part of therapeutical process while their role in diagnosis decreasing. The treatment of thoracic outlet syndrome patients is currently evolving into an interdisciplinary approach integrating angiologists, interventional angiologists and vascular surgeons. This complex multidisciplinary approach improves long term results and quality of life of these patients.