Leiomyosarcomas are exceptional in peripheral veins. Clinical signs are not specific, secondary to effects of the tumoural mass. Computed tomography and MRI give a precise topographic diagnosis. Pathology examination on biopsy or surgical specimens establishes the histological diagnosis. Therapeutic management must take into account local tumour recurrence and metastasis. Surgical exeresis should be wider than the tumour's pseudocapsule to include micrometastases neighbouring the primary tumour. Post-operative radiotherapy (55 to 70 Gy) and chemotherapy should follow. With this management scheme, local disease is controlled in 85% of the cases with a 5-year follow-up. Local or metastatic extension usually occurs within the first 3 years. Management of venous sarcomas thus requires a multidisciplinary cooperation between the surgeon, the radiotherapists and the chemotherapist.