Intravenous leiomyomatosis is a rare benign tumor originating from the uterus that spreads through vessels. In rare cases, intracaval and intracardiac involvement has been described. We report a case of a 64-year-old woman who presented intravenous leiomyomatosis, which originated from the right uterine vein and extended through the inferior vena cava into the right atrium. The tumor was completely removed through a one-stage transabdominal approach with transesophageal echocardiography monitoring. Sparing thoracotomy is important in order to reduce postoperative morbidity and tumor removal by pulling down was technically possible without the risk of embolization.