INTRODUCTION: We present the case of a 37-year old woman diagnosed with intravenous leiomyomatosis (IVL) that was managed uneventfully with multiple-step management.PRESENTATION OF CASE: A 37-year-old woman was admitted because of acute abdominal pain. Emergency Computed Tomography demonstrated a big pelvic mass 5×15cm of heterogenous composition intaking the contrast agent. Total hysterectomy with salpingoophorectomy was proposed to patient, however, patient expressed her will for fertility preservation and gave consent only for the resection of a single ovary. Laparotomy revealed the presence of myoma, multiple lesions of potential adenomyosis and cordon-shaped formations arising from uterus and extending mainly to left ovary. Final histological diagnosis was intravenous leiomyomatosis (IVL). MRI angiography revealed the presence of residual lesions in inferior vena cava. Laparoscopic resection was performed one month after laparotomy and left ovary was resected without complications. Venovenous bypass was finally performed three months later from initial surgery. The process was significantly labored, resulted in the successful resection of intravenous lesions but was complicated intraoperatively by right kidney rupture. After a follow-up of 33 months, case remains uncomplicated without signs or symptoms of potential recurrence.DISCUSSION: Intravenous leiomyomatosis represents a rare clinical entity histologically bening but clinically aggressive. No consensus exists regarding the optimal management, especially in cases with initial will for fertility preservation.CONCLUSION: IVL represents a rare clinical entity often presenting difficulties in diagnosis and optimal treatment. Large case-series studies should be encouraged to assess the optimal management.