Abstract: Objective：To explore the standardized surgical strategy for intravenous leiomyomatosis. Methods：The clinical data of 22 patients with intravenous leiomyomatosis were retrospectively analyzed in Zhongshan Hospital, Fudan University from July 2011 to September 2018. Results：Among the 22 patients, 1 patient underwent two-stage surgery, and other 21 patients underwent one-stage surgery, among whom, 1 patient underwent laparotomy without CPB, 9 patients underwent laparotomy with partial CPB, 10 patients underwent thoracotomy+laparotomy with total CPB, 1 patient underwent laparotomy combined with left intercostal small incision surgery with total CPB. The operative time was 173 to 600 min (［315± 101］ min), and the blood loss was 400 to 9 000 mL (［1 475±1 084］ mL). There were 1 case of perioperative death, 1 case of acute renal injury, 1 case of duodenal fistula, 1 case of coagulation dysfunction, 1 case of thrombosis in inferior vena cava, and the rest patients had no surgical complications. After 2 to 87 months of follow-up, with the median follow-up time of 26 months, 1 patient had pelvic recurrence and benign lung metastasis, 2 patients had pelvic recurrence, and the rest had no recurrence and metastasis. Conclusions：Intravenous leiomyomatosis involves multiple organs. Comprehensive preoperative examination of the relationship between tumors and veins or cardiac cavity is necessary before the surgery. For abdominal and pelvic lesions, hysterectomy and adnexectomy is recommended. For intracardiac leiomyomatosis, complete excision of the lesion under cardiopulmonary bypass is the best surgical strategy. If there is no adhesion between the tumor and vein, one-stage surgery through an abdominal approach is feasible.