Background: Pelvic arteriovenous malformations (AVMs) are rare vascular anomalies that may be congenital or acquired. Acquired pelvic AVMs following pelvic surgery are uncommon and can be challenging to manage, particularly when refractory to endovascular therapy. Case Presentation: We report the case of a 63-year-old woman who developed a large (20 x 12mm), symptomatic pelvic AVM arising from the inferior mesenteric artery. This occurred 14 years following total abdominal hysterectomy with bilateral salpingo-oophorectomy for uterine leiomyoma and subsequent re-laparotomy for another pelvic leiomyoma. Despite five endovascular embolisation procedures, she continued to experience debilitating symptoms related to high output cardiac failure including presyncope, dizziness, exertional dyspnoea, and muscle weakness. She also developed bilateral ureteric obstruction requiring stent insertion. Given the progressive morbidity and mortality risk posed by the AVM, and failure of embolisation, she underwent definitive surgical management with pelvic exenteration, including en bloc resection of the AVM with the left internal iliac arterial and venous system alongside the common and external iliac vein. Histopathological examination confirmed the diagnosis of an arteriovenous malformation. Conclusion: This case highlights pelvic exenteration as a potential definitive treatment option for refractory, symptomatic, acquired pelvic AVMs when endovascular approaches fail. Multidisciplinary evaluation is critical in selecting appropriate candidates for radical surgical intervention.