Intravenous leiomyomatosis (IVL) is a rare benign condition characterized by the proliferation of smooth muscle cells originating from either the uterine venous wall or a uterine myoma. This leiomyomatosis extends most frequently to pelvic vessels, but also occasionally into the inferior vena cava and right cardiac chambers. Preoperative diagnosis is difficult and it should be suspected in the presence of cardiac or pelvic masses in women who have undergone hysterectomy or myomectomy previously. The treatment is hysterectomy, normally associated with a bilateral oophorectomy and removing the mass or metastasis if any. The post-surgical follow-up should be performed at long term and include exploration and imaging, either ultrasound or MRI. The association of antiestrogenic drugs can be useful for disease control, especially in cases where oophorectomy is not performed and the tumor cannot be removed completely.