Objective To explore the clinical characteristics, pathology and treatment of uterus intravenous leiomyomatosis. Methods A total of 6 cases of intravenous leiomyomatosis which confirmed by pathologically were analyzed retrospectively. Results The mean age of 6 patients was 40.1, average times of pregnant and labor were 3 and 1.7. 33.3% patients had a history of uterine-related surgery. Most common symptoms were pelvic mass and abnormal uterine bleeding. None of them was diagnosed preoperative. The pathological manifestation was smooth muscle cell grow along the blood vessels. All patients were diagnosed by postoperative pathology. Over 40 years old patients with diffuse lesions were performed hysterectomy and pelvic tumor resection. Young patients with lesions confirmed to uterus were performed leimyoma resection. The follow-up time was 1-25 months, one patient relapsed (not completely resection). Conclusion Intravenous leiomyomatosis had no specific symptoms, and it mostly affects the women with childbearing age. Pahtology is most valuable for the diagnosis. Radically resection is the main treatment for intravenous leiomyomatosis. All patients need routine follow-up after the operation.