We describe a case of intracardiac leiomyomatosis originating from the uter
us, growing up in the inferior vena cava, and extending into the right vent
ricle. She also found to have lung metastasis. As she declined for further
operative intervention, Tamoxifen was given for the control of disease. Thi
s case represented an unusual rapid recurrence of intravenous leiomyomatosi
s which was potentially preventable. To investigate for the appropriate man
agement, a review of the literature concerning this rare condition was made
.