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Epitheloid haemangiothelioma is extremely rare malignant tumour of vascular origin. Mediastinal location of this neoplasm with large veins involving is not common. We report a case of epitheloid haemangiothelioma in superior vena cava treated by radical resection and superior vena cava replacement with polytetrafluoroethylene prosthetic graft. There is a certain risk of neurological injury during the cross-clamping of superior vena cava in patient with incompletely obstructed veins. Venovenous shunt is a useful surgical strategy in this type of lesion witho