Background: Surgical treatment strategy for acute type A aortic dissection (aTAAD) with mesenteric malperfusion (MMP) is quite challenging as it is often associated with poor patient outcomes, and optimal management strategies remain controversial. Methods: We conducted MEDLINE and EMBASE database searches up to December 31, 2021 for studies on aTAAD with MMP. Data on study design, patient demographics, patient management strategy, mortality, complications, and follow-up were extracted, analyzed, and investigated. Results: Our literature search identified 941 potentially relevant studies, of which 19 were deemed eligible for this study. A total of 352 patients, mean age: 58.4 \pm 11.9 years, diagnosed with aTAAD complicated with MMP were included with an overall prevalence of 4%. Patients for which MMP was observed preoperatively were also included in this analysis. The overall in-hospital mortality amongst these patients was 43.5%, and bowel necrosis and/or multiorgan failure were the major causes of death. Four management strategies for first-line treatment were recognized and these included central aortic repair (191, 54.3%), reperfusion of superior mesenteric artery (SMA) (121, 34.3%), exclusively endo-intervention (11, 3.1%), and exclusively medical intervention (29, 8.2%). These various first-line strategies showed mortality rates of 40.3%, 33.9%, 72.7% and 93.1%, respectively. There was no significant difference in the mortality rate between central aortic repair and reperfusion of SMA as first-line therapies ({\chi }^2 = 1.302, p = 0.254). When compared with central aortic repair and reperfusion of SMA, exclusively medical care exhibited a significantly greater mortality rate (p 0.01). Conclusions: aTAAD complicated with MMP is a rare complication that carries a high mortality rate. Central aortic repair and reperfusion of SMA as first-line treatment strategies appear to be associated with better outcomes compared with exclusively endo-intervention and medical care. Clinical decisions may have introduced biases as no differences were indicated in regards to the way patients were being prioritized for the central aortic repair versus reperfusion of SMA. In regards to variable clinical features and pathology of aTAAD complicated with MMP, an individualized approach is recommended.