Low-grade endometrial stromal sarcoma (LG-ESS) usually exhibits indolent behavior, often misdiagnosed and is associated with a favorable prognosis [1]. Despite the indolent nature of LG-ESS, recurrence is common, the risk is as high as 1 in 3 to 1 in 2 [2]. The behavior of such tumors can lead to invade blood vessels [3]. An intravascular tumor is associated with direct risk of sudden death from pulmonary embolism. There are very few reported cases with clinically obvious intravascular extension. Chemotherapy, radiotherapy and hormone therapy are adjuvant treatments that have not been able to demonstrate a definitive benefit in survival [4]. We want to take advantage of the opportunity, so far unpublished, to demonstrate with this case the surgical challenge described step by step, as well as to demonstrate the importance of surgery as the only curative treatment even in extended intravascular disease [5]. Here in, we present the surgical challenge of a LG-ESS in a 59-year old woman with iliac and cava vein tumoral thrombus extension. We performed a tumoral thrombectomy and vascular repair, with a pelvic exenteration and double barrel wet colostomy. After performing pelvic exenteration, the thrombus with intravascular involvement was addressed at 2 points: inferior cava vein and right common iliac vein with the extraction of the entire thrombus. These defects are corrected by angioplasty and venorrhaphy, respectively. The surgery got a complete cytoreduction. It lasted 290 minutes, with 430 mL blood loss. In patients with LG-ESS, cytoreduction is a reliable option even in extended metastatic disease.