Intravenous leiomyomatosis is an uncommon variant of uterine leiomyomas. These tumors originate from smooth muscle cells of veins or lymphatics. Generally, they can't be diagnosed before surgery. Both gross and histopathological findings are important for diagnosis. Growing into the right ventricle through inferior vena cava may be seen in some of the cases. Benign cystic lung metastases have been also reported. In this article, a 42 year-old woman who had 3 children applied to our outpatient clinic of gynecology with pelvic pain and abnormal menstrual bleeding which had started 7 years ago, is presented. In physical examination uterus was palpated as hard, painful, and abnormally large. Total abdominal hysterectomy and unilateral salphingo-oophorectomy was performed with myomatosis uteri prediagnosis. During operation, frozen section specimen was diagnosed as intravenous leiomyomatosis. By presenting this case, we aim both to emphasize the importance of post-diagnosis follow-up of patients, and also evaluate intravenous leiomyomatosis in the light of literature.