Primary vascular leiomyosarcoma is a rare, aggressively malignant connective tissue tumour, which arises from smooth muscle cells of the vessel walls. This neoplasm involves the extremities in about of 30% reported cases. Preoperative angiograms with CT scans and MRI in conjunction with the clinical signs of vascular and biopsy and immunohistochemical studies are useful tools in the diagnostic and operative planning of intra-vascular leiomyosarcoma.

We report the case of a 34 year-old woman who was referred to us presenting pain, palpable mass in the right inguinal area and tenderness, deep venous thrombosis symptoms and motor dysfunction of her right leg. Biopsy was performed with the diagnosis of Leiomyosarcoma (IIB) of iliac and femoral right veins treated with wide resection and reconstruction of vessels with vascular prosthesis achieving excellent results.

In conclusion, complete surgical resection of the vessel segment is the therapy of choice. Adjuvant therapy, chemotherapy or radiation therapy combined with surgery, is often used for patients with poor prognosis.