Intravenous leiomyomatosis (IVLM) is an unusual neoplasm derived from uterine smooth muscle cells seen in patients with uterine leiomyomas. The typical histological features of IVLM consist of benign smooth muscle cells present within venous vascular spaces of the uterine wall. Increasing intravascular and intracardial spread of IVLM may lead to life-threatening clinical complications. Thorough pathological study of routine hysterectomy specimens may lead to the diagnosis of IVLM. Most affected patients will be cardiologically asymptomatic at the time of diagnosis. Herein, the relatively unknown clinical and morphological aspects of IVLM are presented and discussed.