Low-grade endometrial stromal sarcoma (LG-ESS) accounts for approximately 1 % of malignant tumors in the uterine corpus, grows slowly, and frequently expresses ER and PR. The 5-year survival of patients with primary LG-ESS was shown to be over 90 %. However, approximately 50 % of cases will recur, with recurrence commonly occurring more than 10 years later. Lymph-vascular space invasion is frequently observed, and several cases of LG-ESS with extension into the major vessels have been reported previously. We herein present a case of a 65-year-old Japanese woman diagnosed with recurrent LG-ESS 16 years after receiving primary treatment. Recurrent tumors were located in the left ovary and also in the para-aortic region below the left renal vein, extending into the inferior vena cava and the right atrium through the left renal vein and left lumbar segmental veins. We judged primary total resection of all tumors to be impossible and described the surgical procedure, risks associated with removing intracaval and intracardiac tumor thrombus, and hormone therapy in this case conference.