Intravenous leiomyomatosis is a rare tumor described only in women, in which benign smooth muscle cells grow into the pelvic venous channels. The tumor may cause life-threatening symptoms if there is involvement of the inferior vena cava or the right atrium. This is a case study of intravenous leiomyomatosis with left ovarian extension in a 42 years old woman. The patient was admitted for total abdominal hysterectomy due to uterus myomatosus and menorrhagia. During the operation a frozen section was sent for examination revealing intravenous leiomyomatosis. Thereupon, bilateral salpingo-oophorectomy was also performed. This article reviews the cause, disease, presentation, diagnosis, treatment and recurrence of intravenous leiomyomatosis.