Preoperative renal dysfunction is associated with mortality in patients with acute type A aortic dissection (ATAAD) repair. However, the long-term outcome of chronic kidney dysfunction (CKD) in ATAAD is unclear. The study aimed to evaluate the long-term outcome of CKD in patients with ATAAD repair. We retrospectively studied patients with ATAAD repair using data from the Taiwan’s National Health Insurance Research Database between July 1, 2004, and December 31, 2013. The outcomes of interest included all-cause mortality, readmission due to any cause, redo aortic surgery, major adverse cardiac and cerebrovascular events, and liver and renal outcomes. There were 3328 patients who received ATAAD repair. These patients were divided into CKD and non-CKD groups. In-hospital mortality in the CKD group was significantly higher than that in the non-CKD group (32.5% vs 18.8%, respectively, odds ratio 2.14, 95% confidence interval [CI] 1.37–3.36). During long-term follow-up, patients with CKD had higher risks of all-cause mortality including in-hospital death (52.6% vs 32.5%; hazard ratio 1.83, 95% CI 1.32–2.55), mortality after discharge (29.7% vs 16.8%; hazard ratio 2.09, 95% CI 1.02–4.29), and readmission rates (67.1% vs 51.6%; subdistribution hazard ratio 2.00, 95% CI 1.43–2.79). However, no significant difference was observed between the dialysis and non-dialysis groups. On the basis of our results, patients with CKD carry a poor long-term outcome after ATAAD repair. Cardiac surgeons should be aware of this condition when dealing with ATAAD repair.