Intraventricular smooth muscle disease is a rare neoplastic condition. The tumor originates in the uterus or in the venous wall of the extrauterine pelvis. The tumor may protrude into the venous channels of the uterus or pelvis and extend and expand to the inferior vena cava via the iliac or ovarian veins. If the tumor extends into the right atrium, or even into the right ventricle and pulmonary artery via tricuspid valve, it may cause serious circulatory disorders and syncope or sudden death. Surgical resection of the primary tumor, vena cava and right heart system tumor is the best treatment. So far, there are more than 100 cases of this tumor involving the heart reported in the literature. Since January 2001, our department has treated more than 10 cases of intraventricular smooth muscle tumor involving the heart, and the primary lesion was removed at the same time or in stages through multidisciplinary collaboration, and all of them obtained satisfactory results with no recurrence in postoperative follow-up. In 1896, Hirschfeld first described IVL (intravenous smooth muscle tumor disease), and in 1907, Durck reported the first case of IVL involving the heart. It is often misdiagnosed as primary cardiac tumor or blood clot. IVL should be highly suspected in patients with uterine fibroids (unoperated/operated) combined with lower extremity venous thrombosis or with cardiac involvement, and should also be considered in gynecologic surgery when finger-like protrusions from the uterus or worm-like growths into the parametrial broad ligament or other pelvic organs are detected. The imaging diagnosis can be made by a combination of abdominal ultrasound and cardiac ultrasound, enhanced CT, MRI, and can provide Adequate information for surgical design. MRI is generally considered to be superior to CT because it not only clarifies the extent of the lesion, but also shows the characteristics of the tumor within the vessels, such as whether there are adhesions with the vessel wall and the site of adhesions; it is also a guide to determine the scope and method of surgery. However, CT is more commonly used in clinical practice because of its low cost and high popularity. Based on the data of this group, we believe that CTV can effectively and conveniently monitor the recurrence and growth of tumor after surgery. The condition of abdominal tumor can be clarified by abdominal ultrasound. Only a few cases still require inferior vena cava and right atrial angiography. In the diagnostic process, the disease needs to be differentiated from mucinous tumors, Buga syndrome, thrombosis in the inferior vena cava, as well as uterine smooth muscle sarcoma, renal cancer and hepatocellular carcinoma extending into the inferior vena cava. In 1974, Mandelbaum reported the first case of surgical treatment of IVL involving the heart, so surgery has become the best treatment method. The aim of surgery is to remove the tumor in the heart and inferior vena cava on the one hand, and to remove the primary lesion to prevent recurrence on the other. The choice of surgical plan should be based on the primary lesion, the extension of the tumor in the vein and the heart cavity, and previous surgical experience. The ideal surgical plan is to operate in one stage to avoid complications such as tumor embolism, tumor development and hemodynamic disturbances during the interoperative period and to reduce the risk of reoperation. In recent years, with the accumulation of experience in the treatment of this disease, the deepening of the understanding of pathological anatomy, and the advancement of extracorporeal circulation technology, more and more operators adopt one-stage surgery for radical treatment. However, for patients with difficult and severe disease and a history of multiple previous surgeries resulting in tight local adhesions at the primary site, one-stage surgery may present high-risk factors such as excessive operating time and large trauma bleeding, and staged surgery is still a safe choice. Since one of the biological characteristics of uterine smooth muscle tumors is their slow growth, close follow-up between surgeries, appropriate medication, and early reoperation can minimize the corresponding risks. The complexity of surgery varies greatly. Some of these tumors are thin in diameter, with a striated tissue structure floating in the lumen like watercress, and only the root is fixed in the internal iliac vein or the genital vein, so surgical resection is relatively easy, and there are some reports of tumor removal via the right atrium. However, there are some cases in which the tumor has not yet caused inferior vena cava obstruction, but there is a risk of local adhesion to the vessel wall. At this time, forceful traction on the tumor may cause local vessel wall damage, resulting in retroperitoneal hematoma and even risk of cardiac rupture. In addition, some scholars (Harris LM et al.) have reported the removal of intraventricular smooth muscle tumor disease extending into the right cardiac cavity in one stage without extracorporeal circulation via the abdomen. To avoid the risk of pulmonary artery embolism, we do not recommend the extraction of the tumor solely from the abdominal incision; therefore, even in cases where the tumor is not large in diameter, we recommend a combined thoracoabdominal incision to expose the entire inferior vena cava, deep hypothermic stop circulation under extracorporeal circulation, and removal of the intracardiac and inferior vena cava tumor after thorough treatment of the root of the tumor. The gross pathology shows a long tumor tissue extending from the iliac vein to the right heart with intact continuity, no adhesion and invasion to the vena cava and cardiac cavity, and only adhesion to the end of the internal iliac vein, and the tumor is less than 1 cm in diameter at the end of the internal iliac vein. therefore, this tumor only adheres to the internal iliac vein and can be easily extracted from the right atrium intraoperatively. Extraction is also not indicated for tumors originating from the genital vein because of the risk of causing rupture of the renal vein, leading to uncontrollable bleeding and requiring nephrectomy in severe cases. In large tumors it can cause complete obstruction of the inferior vena cava, with endovascular as well as endocardial adhesions, making the procedure very difficult. In the former case, however, even if this type is free throughout, it may adhere to the vein wall during growth along the vessel, so intraoperatively avoid transvenous right atrium and perform tumor resection under deep hypothermic stop circulation instead. It is suggested that deep hypothermic stopped circulation is also not necessary. Lam reported that approximately 70.6% of IVLs were completely resected, of which 60.4% were treated with staged surgery and the remaining 39.6% with stage I surgery. In patients with a genital vein origin or bilateral iliac vessels, large tumor diameter, significant obstruction of vena cava flow, and clinical manifestations of right heart failure, tumor resection should be performed under direct vision. Because such tumors involve a wide range, the preoperative relationship between the lesion and multiple organs in the pelvis and abdominal cavity, especially kidney, ureter and liver tumors, must be fully evaluated. For intraoperative localization to avoid ureteral injury, we recommend routine preoperative placement of a Double-J tube in the ipsilateral ureter. In addition, intraoperative preparation of the vena cava and renal vein reconstruction and preparation of autologous pericardial slices or corresponding vascular patches are required. In addition, the presence of estrogen receptors in intravascular smooth muscle tumor tissues can contribute to tumor growth and recurrence under the effect of estrogen in vivo, thus also affecting its prognosis. The relevant literature reports that estrogen-suppressing hormone therapy has some therapeutic value in patients with incompletely resected tumors. In conclusion, intraventricular smooth muscle tumor is a rare benign tumor. Once ILV extends into the heart chambers, it can lead to sudden death, and timely surgery is the best treatment. A thorough preoperative examination and preparation is the key to surgical cure of this disease, and the diagnosis and treatment of this disease requires a multidisciplinary approach.