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Intravenous leiomyomatosis(IVL)is a rare benign neoplasm，characterized by benign smooth muscle growth in vein that can extend to the heart cavity. The literature include only 100 cases with cardiac involvement. This tumor is diagnosed frequently from symptoms that result from intravenous or intracardiac obstruction after hysterectomy.We describe an additional case of intravenous leiomvomatosis extending to the right ventricle outlet tract of the heart through the left internal iliac vein and the inferior vena cava，which was removed successfully in a surgical procedure.