Background: Atypical leiomyomas are relatively uncommon in the general practice of gynaecology. We present a case of a large uterine bizarre leiomyoma removed by operative hysteroscopy and review of the literature. Case: The patient, a 49-year-old, gravida 3, para 3, perimenopausal Greek woman presented to our Department because of dysmenorrhea and abnormal vaginal bleeding. She underwent hysteroscopy in which a large submucosal leiomyoma was detected and entirely removed in one session. The histopathology revealed bizarre uterine leiomyoma. Discussion: There is no evidence to indicate that hysterectomy is necessary, if the diagnosis of atypical leiomyoma has been firmly established.