question: Low-grade endometrial stromal sarcoma with intracaval and intracardiac extension represents a complex and often lethal condition. A case of a 40-year-old woman, who underwent hysterectomy for endometrial stromal sarcoma 4 years previously and developed recurrence with intracaval and intracardiac metastases, is presented. Operative management of resection of the intracaval and intracardiac tumor is described. The literature is reviewed in order to highlight the data on diagnosis and management of recurrent endometrial stromal sarcoma with vascular involvement, including the multimodality approach required in treating this disease.