Abdominal aortic aneurysm (AAA) with concomitant horseshoe kidney (HSK) is a rare clinical entity. Special consideration to the abnormal anatomy of the kidney, such as the isthmus of the HSK straddling the aneurysm and the accessory renal arteries originating from the abdominal aorta, should be taken during its surgical treatment. Current treatment methods include open surgery (transabdominal or retroperitoneal), endovascular repair and hybrid surgery. But the indications and specific technical approach for those procedures remain inconclusive. In this paper, the authors present and analyze the essentials and difficulties in surgical treatment of this condition, so as to provide a reference for clinical practice.