Intravenous leiomyoma with intracardiac extension is a rare entity that necessitates surgical excision to alleviate symptoms and prevent life-threatening complications. These procedures are generally performed under cardiopulmonary bypass, and review of the literature reveals an evolution in management of this disease from 2-stage to single-stage procedures. We report the case of a 45-year-old woman with an intravenous leiomyoma with intracardiac extension after presenting with syncopal symptoms. The intravenous tumor and its supradiaphragmatic extension were ultimately resected via laparotomy without the need for cardiopulmonary bypass. Distal control was achieved by passing an occluding balloon catheter above the tumor through a venotomy with fluoroscopic guidance and echocardiography, thus enabling protected tumor extraction through the intra-abdominal inferior vena cava. In selected patients, we believe use of endovascular techniques combined with proper preoperative imaging can safely permit single-stage resection of these tumors via laparotomy without cardiopulmonary bypass.