Objective Hyperbilirubinemia is one of the common complications after cardiac surgery and is associated with increased mortality. However, to the best of our knowledge, the report on clinical significance of postoperative severe hyperbilirubinemia in Stanford type A aortic dissection (AAD) patients is limited. Therefore, the purpose of our present study is to assess the characteristics and outcomes of AAD patients with post-operation severe hyperbilirubinemia.Methods Patients who underwent surgical treatment for AAD in our center between January 2015 and December 2018 were retrospectively screened. In-hospital mortality, long-term mortality, acute kidney injury (AKI), and the requirement of continuous renal replacement therapy (CRRT) were assessed as endpoints. Univariate and multivariate regression models were employed to identify the risk factors of these endpoints.Results Of the 2210 screened patients, 271 (12.3%) were included. Of the included patients, 222 (81.9%) experienced postoperative AKI, and 50 (18.5%) received CRRT. In-hospital mortality was 30.3%. The 1-year, 2-year, and 3-year cumulative mortality were 32.9%, 33.9%, and 35.3%, respectively. Multivariate Logistic regression analysis indicated that age ( P < 0.033), AKI stage 3 ( P < 0.001), the total amount of blood transfusion after surgery ( P = 0.019), mean arterial pressure (MAP) in the first postoperative day ( P = 0.012), the use of extracorporeal membrane oxygenation (ECMO) after surgery ( P = 0.02), and the peak total bilirubin (TB) concentration ( P = 0.023) were independent risk factors of in-hospital mortality. The optimal cut-off value of peak TB on predicting in-hospital mortality was 121.2 μmol/l. Older age, high preoperative serum creatinine (SCr) concentration, and prolonged cardiopulmonary bypass (CPB) time were identified as the independent risk factors of AKI. High preoperative SCr concentration was identified as the only independent risk factor of the requirement of CRRT.Conclusions Post-operation severe hyperbilirubinemia is a common clinical presentation in AAD surgery patients. Post-operation severe hyperbilirubinemia AAD patients with older age, lower MAP, increased blood transfusion, stage 3 AKI, the use of ECMO, and the increased peak TB had higher risk of in-hospital mortality.