Background: Patients with connective tissue diseases are at high lifetime risk of developing thoracic aortic aneurysms (TAAs) due to defects in extracellular matrix composition which compromise the structural integrity of the aortic wall. It is vital to identify and manage aneurysms early to prevent fatal complications such as dissection or rupture. Method: This review synthesises information obtained from a thorough literature search regarding the pathophysiology of TAAs in those with heritable connective tissue diseases (HCTDs), the investigations for timely diagnosis and current operative strategies. Results: Major complications of open repair (OR) include pneumonia (32%), haemorrhage (31%) and tracheostomy (18%), with a minor risk of vocal cord paresis (9%). For thoracic endovascular aortic repair (TEVAR), high rates of endoleak were documented (38‐66.6%). Reintervention rates for TEVAR are also high at 38‐44%. Mortality rates were documented as 25% for open repair and vary from 14% to 44% for TEVAR. Conclusion: OR remains the mainstay of surgical management. While TEVAR use is expanding, it remains the alternative choice due to concerns over endograft durability, limited long‐term outcome data and the lack of high‐quality evidence regarding its use in HCTD patients. [ABSTRACT FROM AUTHOR]