Aortic root aneurysm is a frequent condition, which is associated with increased mortality and morbidity compared to other aortic aneurysms. The treatment relies on surgery, most often with the Bentall procedure or aortic valve-sparing root replacement (AVSRR). AVSRR is a highly specialised treatment associated with increased survival and low incidences of valve-related complications compared to the Bentall procedure. The purpose of this review is to highlight the diagnosing and surgical treatments of AAR with a focus on the newest international experiences with AVSRR.