Intravascular leiomyomatosis (IVL) is a benign pathology of the uterus. It is characterized by a leiomyocytes proliferation in the lumen of the myometrial and pelvic veins. Prognosis is related to its hormonal dependence and its potential vascular extension. Surgical excision must be complete to avoid recurrence’s risk. In the light of a 31-year-old nulliparous patient case report, with a pelvic mass developed at the uterus, which histological examination confirmed the IVL diagnosis, we will discuss the clinical, evolution, prognosis and management features of this rare variety of myoma.