The association between aortic pathology and congenital renal fusion adds little morbidity or mortality to the natural history of either entity. Nevertheless, it complicates the management of aortic pathology, specifically degenerative atherosclerotic disease. The therapeutic goal is to resect the aneurysm or bypass the occlusive segment while preserving renal function. Important principles in the management of these difficult patients include accurate preoperative detection and characterization of the renal abnormality, complete familiarity with the likely anatomic variations associated with this anomaly, adequate exposure of the aortic lesion while preserving renal perfusion, and avoidance of injury to a frequently abnormally located collecting system. In this review we report two cases of abdominal aortic aneurysm associated with congenital renal fusion. The principles of management are thoroughly discussed, and guidelines aimed at facilitating decisions made during the clinical treatment of these difficult patients are suggested.