Intravenous leiomyomatosis is a rare benign smooth muscle tumor arising from uterine muscle or vascular wall. The disease grows into pelvic venous channel, and can extend to inferior vena cava, and, in exceptional cases, right side of the heart. A case of intravenous leiomyomatosis is presented with a brief review of literatures. A 42-year-old woman presented with one month history of vague right lower quadrant pain and leg swelling. Six years before admission, she had undergone total abdominal hysterectomy for leiomyoma of the uterus and excision of recurred mass and right salpingo-oophorectomy 32 months ago. During the follow-up, right adnexa mass was detected and the size grew progressively, so transferred to our hospital. CT scan and venograpy revealed out the large pelvic cavity mass and intravenous extension to right common iliac vein, bifurcation of IVC. Preoperative tumor embolization followed by resection of recurred pelvic tumor, removal of intravenous tumor via internal iliac vein venotomy and left salpingo-oophorectomy were done. After the operation, the symptoms were relieved and the patient resumed her normal activity.