Objective To summarize the imaging features and clinical treatment experience of intravascular leiomyomatosis. Methods The clinical manifestations, imaging data, surgical treatment, postoperative pathology and immunohistochemical staining of 5 patients with intravascular leiomyomatosis diagnosed in our hospital from Jan. 2014 to Dec. 2019 were retrospectively analyzed, and the imaging features and clinical treatment experience were summarized. Results The 5 patients were all females, with an average age of 51.8 years (ranged 46-60 years), and all had a history of hysteromyoma, and 2 of them had a history of hysterectomy and bilateral adnexectomy. Two cases presented with leiomyoma invading the right atrium, showing symptoms of impaired cardiac function; the remaining 3 cases presented with right lower limb swelling, abdominal masses, and lower abdominal distension. Four cases underwent ultrasound and computed tomography (CT) angiography of the inferior vena cava, 2 cases underwent magnetic resonance imaging (MRI) plain scan + enhanced examination, and 1 case underwent angiography. All patients underwent angiomyoma resection+angioplasty. Of the 2 cases presented with leiomyoma invading the right atrium, 1 case underwent primary surgery, 1 case underwent secondary surgery, and both patients recovered well after surgery. One case was re-operated due to recurrence 4 years after surgery. One case underwent a major resection of the tumor + ligation of the common iliac vein due to excessive bleeding during the operation, and the tumor in right common iliac vein was not resected. In the 5 cases, the common features of immunohistochemical staining were smooth muscle actin (SMA) (+), calponin (CALP) (+), P53 (-), CD31 (-), Ki-67 (-); only 1 case was positive for estrogen receptor (ER) and progesterone receptor (PR), 1 case was partially positive for both ER and PR, 1 case was partially positive for ER and slightly positive for PR, and 2 cases had no ER or PR staining data. All the 5 patients were followed up regularly and no recurrence was found until Feb. 2021. Conclusion The clinical manifestations of intravascular leiomyomatosis are not specific, but the imaging features are clear. The diagnosis accuracy can be improved by combining clinical manifestations and gynecological history.