Intravenous leiomyomatosis with a cardiac extension is an extremely rare condition. In this report, a case of a 43-year-old female patient is described: she was operated for right atrial mass protruding into the inferior vena cava, which was later diagnosed as leiomyoma. After a 3-year symptom-free period, recurrence of the extension through the inferior vena cava was observed. After abdominal ultrasonographic examination, which revealed bilateral ovarian and retroperitoneal mass, bilateral oopherectomy, retroperitoneal, and right atrial mass excision was done. The retroperitoneal and right atrial mass was reported as leiomyoma. On her last admission, she had complaints of dizziness, abdominal pain, and bilateral leg edema; and right atrial mass extending through the common iliac vein was noted, but the patient did not accept any further treatment modality.