The contemporary data regarding the role of the thoracic endovascular aneurysm repair (TEVAR) in patients with connective tissue disorders (CTD) are reviewed. We performed search of the data in electronic databases PubMed, SCOPUS, Embase, Google Scholar OVID, related to the use of TEVAR. Currently, there is a limited amount of large cohort size studies outlining the use of TEVAR in patients with CTD. Endovascular prosthetics of the thoracic aorta in patients with accidents is dangerous because of the progressive dilatation of the aorta and the high probability of further re-interventions in later life. The analysis showed that open reconstruction still remains a gold standard of intervention in young patients with progressive CTDs, especially in acute type of aortic dissection. However, long-term data need to be published to support this practice.