Intravenous leiomyomatosis (IVL) is a rare neoplasm that is characterized by a histologically benigh-looking smooth muscle cell tumor growing within the uterine and the extrauterine venous system. We report here on patient had a uterine and extrauterine leiomyoma that extended into the right atrium through the inferior vena cava. A 19-year old female patient was suffering from dyspnea, swelling of the lower extremity, abdominal pain and abdominal distension. She had total abdominal hysterectomy performed at a private clinic due to uterine leiomyoma 4 months previously. 4 months after the first operation, we again completely excised the recurred intraperitoneal tumor mass. At 6 months after the re-operation, the tumor mass recurred intraperitoneally. A preoperative abdominal CT scan and an echocardiogram revealed multiple tumor masses that were located intraperitoneally and they extended to the right atrium. We performed intraperitoneal tumor excision and removal of the intravenous tumor mass via the right iliac vein by the one-stage approach. At present, the patient has shown a clinically favorable outcome except for local recurrence of tumor mass in the pelvic cavity. The cure of this complex disease emphasizes the need for a planned systemic approach by a multidisciplinary surgical team. We present here an unusual case of intravenous leiomyomatosis that originated from the uterus and it extended to the inferior vena cava and right atrium.