Intravenous leiomyomatosis is considered to be a rare neoplastic disease usually arising from uterine fibromyomata, but its true incidence may be under-recognized. Mortalities from this condition are usually the result of intracardiac involvement. A case report of successful treatment of intravenous leiomyomatosis with intracardiac extension is presented, together with a review of the existing literature concerning this rare condition.