Introduction: Abdominal aortic aneurysm (AAA) is a frequent vascular pathology, especially in elderly males. Horseshoe kidney (HSK), the most common renal fusion anomaly, presents a surgical challenge when associated with AAA. We report a case of successful open repair of a juxtarenal AAA in a patient with HSK. Case Presentation: A 68-year-old male with hypertension, diabetes, and active smoking was diagnosed with a 60 mm juxtarenal AAA and horseshoe kidney during routine imaging. The patient underwent elective open repair via a retroperitoneal approach. A bifurcated aorto-bi-iliac graft was placed. The right renal artery was reimplanted, and an inferior polar artery was revascularized using a saphenous vein graft. Discussion: HSK complicates AAA surgery due to anomalous renal vasculature and anteriorly located isthmus. Open repair allows precise revascularization and direct access to the aorta. Renal preservation strategies are crucial to reduce postoperative complications. Conclusion: Open surgical repair remains a reliable option in AAA with HSK when renal revascularization is necessary. Thorough preoperative planning and tailored reconstruction are key to achieving optimal outcomes.