Objective: To describe the case of rare sarcoma of the inferior vena cava and the literature review. Case report: 58-year old man with non-specific symptoms was admitted to hospital. Correct preoperative diagnosis of leiomyosarcoma of the inferior vena cava in its infrarenal part was established using ultrasound and spiralCTexamination. Inferior vena cava flebography confirmedintraluminal portion of the leiomyosarcoma and collaterals. The patient was treated with tumor extirpation, vena cava resection and adjuvant radiotherapy. At present, the patient is 18 monthsmetastases and local recurrence free. Conclusion: The leiomyosarcoma is rare entity with poor prognosis. Delayed diagnosis is due to its slow growth and non-specific symptoms. Nowadays spiral CT including CT flebography and magnetic resonance are accepted methods in the diagnostic algorithm for this rare neoplasia.

        Key words: leiomyosarcoma - inferior vena cava - spiral CT