Background: Uterine arteriovenous malformations (AVM) are extremely rare entities, with less than 100 cases reported in the literature. Synonyms for AVM are arteriovenous fistula, branched aneurysm, hemangioma, pulsating angioma, and cavernous angioma. Incidence of uterine AVM according to the studies of O´Brien et al., who identified uterine AVM in 21 women based on 464 pelvic ultrasound examinations for uterine bleeding, reported an incidence of 4.5%. However, Yazawa et al. examined 959 patients prospectively and observed an incidence of 0.6% of uterine vascular malformation on ultrasound examination The etiology of uterine AVM can be congenital or acquired. The purpose of the study was to raise awareness on the existence of these injuries and understanding their risk factors, to study different treatment methods, especially conservative or minimally invasive ones, as well as methods for diagnosing of these malformations. The following key words were used as a search engine: uterine arteriovenous malformation, uterine malformation, circoid aneurysm. Only full-text articles were analyzed. Conclusions: Uterine AVM is a rare cause of uterine bleeding. However, it is a potentially life-threatening disorder in which patients present with vaginal bleeding that may be profuse and cause hemodynamic instability.