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Purpose – Intravenous leiomyomatosis (IVL) is a very rare subtype of leiomyoma, involving the right obstruction of the heart, and is an unusual cause of outflow tract obstruction. The IVL grows from the vessel’s smooth muscle, protruding into the vessel’s lumen, and can expand to the right atrium and even beyond causing death due to blood flow obstruction into the right atrium and even to pulmonary artery.

Design/Methodology/Approach – We present a 33-year-old Indonesian woman with cardiopulmonary symptoms predominantly, and marked by an intravascular leiomyoma extending from inferior vena cava (IVC) to right chamber atrium, with the chief complaint being easily fatigued since seven months.

Findings – Echocardiography observations found a mass in the right atrium and the IVC that caused dynamics obstructed in tricuspid valve, right atrium and ventricle were dilated, no left ventricular (LV) hypertrophy, normal LV and right ventricular (RV) functions, and no valve abnormality except the tricuspid valve gradient being 21 mmHg with mild regurgitation. Venography resulted in a mobile tumor mass in IVC mouth which partially flew into the right atrium, and partially blocked the IVC mouth. Tumor size was 6.4 cm × 4.8 cm. Abdominal multislice computed tomography resulted in a residual soft tissue mass (leiomyoma) along the IVC extended to the right atrium. The tumor mass size in the IVC and the right atrium was bigger compared to tumor mass on July 2008. The correct diagnosis was established during surgery; therefore a two-stage resection was done.

Originality/Value – Surgical resection is the best treatment for intracardiac extension of intravenous leiomyoma. To remove the ilio-caval portion, iliac venotomy was recommended for the tumor in both stages of the surgeries.