We report a case of intravenous lobular capillary hemangioma in the subclavian vein, causing venous thoracic outlet syndrome. A 32-year-old woman was referred to our unit with facial and left arm oedema. Ultrasound evaluation, computed tomography and magnetic resonance imaging showed a hypervascular mass in the middle portion of the subclavian vein, with arrest of venous flow. Through an infraclavicular approach, we excised the venous axis with the endovascular palpable tumour that extended from the axillary-subclavian junction to the jugular-subclavian junction, without reconstruction. The postoperative period was uneventful. The patient recovered well without recurrence at one year from surgery.