Imaging evaluation of uterine masses is important to assess the type of lesion and to target surgery, if surgical excision is necessary. This can be decisive in fertile women with benign masses resembling malignancies, in order to avoid overtreatment. In this study, the magnetic resonance imaging (MRI) appearance of cotyledonoid dissecting leiomyoma of the uterus, a rare benign variant of leiomyoma mimicking malignancy, is presented.