Whereas uterine leiomyoma is a common woman disease, intravenous leiomyomatosis with intracaval and intracardiac complications is a rare condition. The initial presentation is dependent upon the severity of the intracardiac involvement, although complete surgery is the best treatment. The case of a 39-year-old woman is described here, with an initial presentation of dyspnea and right heart failure. Leiomyomatosis originating from the uterus and extending to the inferior vena cava and right atrium was diagnosed from various preoperative studies. The patient was resuscitated because of respiratory failure and severe right heart failure. However, she was operated successfully through a two-stage approach and remained well postoperatively. This case illustrates an intriguing presentation of intravenous leiomyomatosis and a curative surgical intervention even in critical condition.