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Fenestrated endovascular abdominal aortic aneurysm repair with concomitant horseshoe kidney. Horseshoe kidney, representing abnormal fusion of the inferior renal poles, is a rare anatomic anomaly posing challenges in the setting of surgical abdominal aortic aneurysm repair. Historically, open repair has been the favoured surgical approach. However, due to the location of the renal isthmus and wide-ranging variation in anomalous renal vasculature, endovascular aneurysm repair (EVAR) has emerged as a popular, less invasive alternative. We describe one of the first published cases of two-fenestration EVAR in a patient with concomitant horseshoe kidney, followed by a discussion of current trends in surgical management. With the increasing availability to customise fenestrated grafts to patients' unique anatomy, this advanced EVAR technique may emerge as the preferred approach in certain cases.