Presentation of one case of inferior cava leiomyosarcoma in a 24-year old female patient, incidentally diagnosed after performance of ultrasound. The complementary examinations performed (CAT, NMR, arteriography) guided to a mass of suprarenal origin. During surgery, a tumour of the inferior cava vein was suspected and was later confirmed through the pathoanatomical study of the surgical piece. Review of the clinical and diagnostic aspects placing special emphasis on treatment.