Intracardiac leiomyomatosis (ICLM) is a rare tumor that usually originates from the mesenchymal cells of the uterus. If left untreated, it may lead to cardiac symptoms and sudden death. The present case was a 45-year-old female patient who presented with occasional palpitations. The imaging methods revealed a heterogeneous mass likely to be leiomyoma with intense enhancement in the arterial phase in the uterus, extending from the ovarian and uterine veins to the heart through the inferior vena cava. The tumor council attributed these findings to intravenous leiomyomatosis with intracardiac extension. Accordingly, a multidisciplinary surgical team performed complete excision of the intracardiac leiomyomatosis in the patient presented herein using only the laparotomic approach. The gold standard in the treatment of ICLM is complete tumor excision. Excision of intracardiac tumors can be performed through controlled traction from the abdominal region.