Intravascular leiomyomatosis is a rare condition characterized by histologically benign smooth muscle cell growth within the uterine veins and inferior vena cava and, rarely, into the right chambers of the heart. A 39-year-old female with a history of hysterectomy for uterine leiomyoma was admitted for lower limb oedema and progressive dyspnea of 5 months’ duration. Transthoracic echocardiography and contrast-enhanced computed tomography showed a large mass extending through the inferior vena cava into the right chambers of the heart. As there was a suspicion of an intravenous thrombus, the patient underwent surgical resection. The histopathology of the resected mass led to a diagnosis of Intravascular leiomyomatosis with intracardiac extension.