Objective and importance: Cases of intraorbital arteriovenous malformations are rare, and their management presents a challenge for multidisciplinary teams.
Clinical presentation: A 39-year-old woman developed a pulsating exophathalmos, visual loss, and ocular pain within 1 month, revealing the presence of intraorbital arteriovenous malformations. She underwent only surgical treatment.
Intervention: A fronto-orbital approach with removal of the orbital rim was performed. A CO2 laser facilitated all the surgical steps.
Conclusion: Full recovery of vision and correction of the proptosis have been achieved, and postoperative magnetic resonance imaging and angiography confirmed the total removal of the malformations. The literature is reviewed regarding the management of such lesions. Surgery remains the treatment of choice. The role of embolization is discussed.