Rationale: Although surgical treatment strategies for patients with extensive thoracic aortic disease involving the aortic arch have improved considerably, the impact of stent graft length and placement site on aortic remodeling at long-term follow-up is not fully understood, and the protection of the Adamkiewicz artery (AKA) using the frozen elephant trunk (FET) method is also unclear.Patient concerns: The patient was a 69-year-old man with diabetic nephropathy who became increasingly fatigued and started maintenance hemodialysis 6 months prior to admission. At 64 years, he underwent clipping of a right cerebellar artery aneurysm. In addition, a 1.8 cm aneurysm was found in the contralateral extracranial internal carotid artery. He also had an atrial septal defect and moderate aortic regurgitation and was receiving continuous positive airway pressure therapy for sleep apnoea syndrome.Diagnosis: He had aneurysms in the aortic arch (4.8 cm in diameter) and descending aorta (6 cm in diameter), which was located at T6-9. Preoperative 3-dimensional computed tomography showed that the (AKA) bifurcated at T10-11.Interventions: Considering the patient's several comorbidities and frailty, we planned to perform 1-stage extended aortic arch repair using the FET procedure. However, we performed 2-stage aortic surgery to prevent spinal ischemia, anticipating substantial cardiac enlargement and blood pressure instability due to dialysis treatment. Aortic valve replacement, atrial septal defect patch closure, and aortic arch surgery were performed. A 7-cm elephant trunk was inserted in the descending aorta. Postoperatively, the patient continued rehabilitation until his blood pressure stabilized during dialysis therapy. At postoperative week 4, he underwent thoracic endovascular aortic repair for a descending aortic aneurysm.Outcomes: After surgery, his physical strength decreased; however, he recovered and was discharged 1 month later without any complications. One year after the second operation, he is living a healthy life.Lessons: Extensive aortic arch surgery using the FET procedure is effective for distal aortic arch and descending aortic aneurysms. Nevertheless, in cases in which the position of the AKA is close to the aortic aneurysm and blood pressure control is difficult, a 2-stage procedure and accurate positioning of thoracic endovascular aortic repair are both desirable.