This systemic review of the literature and meta-analysis aimed to evaluate the current state of the evidence for and against reimplantation of the aortic valve (RAV) versus the composite valve graft (CVG) intervention in patients with Marfan syndrome. Random effects meta-regression was performed across the study arms with logit-transformed proportions of in-hospital deaths as an outcome measure when possible. Results are presented as odds ratios with 95% confidence intervals (CIs) and P-values. Other outcomes are summarized with medians, interquartile ranges (IQR) and ranges and the numbers of patients at risk. Twenty retrospective studies that included a combined 2156 patients with long-term follow-up were identified for analysis after a literature search. The in-hospital mortality rate favoured the RAV procedure with an odds ratio of 0.23 [95% CI 0.09-0.55, P = 0.001]. The survival rate at mid-term for the RAV cohort was 96.7% (CI 94.2-98.5) vs. 86.4% (CI 82.8-89.6) for the CVG group and 93.1% (CI 66.4-100) for the RAV group vs. 82.6% (CI 74.9-89.2) for the CVG group for the long term. Freedom from valve-related reintervention (median percentages) for the long term was 97.6% (CI 90.3-100%) for the RAV procedure and 88.6% (CI 79.1-95.5) for a CVG. This systematic review of the literature stresses the advantages of the RAV procedure in patients with Marfan syndrome in regard to long- and short-term results as the treatment of choice in aortic root surgery. The RAV procedure reduces in-hospital as well as long-term deaths and protects against aortic valve reintervention.