Background. Ovarian fibromatosis is a very rare nonneoplastic disease. Due to the rarity and atypical clinical presentations, they may give rise to a misdiagnosis of malignancy and unnecessary extensive surgical interventions. Literature lacks definitive data about this rare disease and its preoperative evaluations. MRI together with the intraoperative frozen section may help us to define the benign nature of the disease. In this report, we aimed to review the literature and give a highlight to the gynecologic oncologists about this rare disease.