Kommerell's diverticulum is a developmental error with a remnant of fourth dorsal aortic arch, named after Dr. Kommerell, a radiologist, who made the first diagnosis in a living individual. The diverticulum can occur in both the left and right aortic arch, from which an aberrant subclavian artery rises to the contralateral side. Pediatric patients often present with airway symptoms whereas dysphagia and chest discomfort are more common in the adult patients. Computed tomography or magnetic resonance imaging can provide details of the diverticulum, associated arch anomalies, and its relationship with surrounding organs. Recent histological studies indicated the presence of cystic medial necrosis in the diverticulum wall, which would explain the reported high rates of aortic dissection and rupture associated with the diverticulum. Accumulated knowledge on this entity and the recent advancement of imaging techniques, surgical/endovascular strategies, and perioperative management, have led to more aggressive intervention to the diverticulum in the early phase. While still under debate it is generally accepted to consider surgical intervention when the diameter of the diverticulum orifice exceeds over 30 mm, and/or the diameter of the descending aorta adjacent to the diverticulum exceeds over 50 mm. Treatment options include open surgical repair, hybrid endovascular repair, and total endovascular repair. The selection of treatment strategy for Kommerell's diverticulum should be based on the anatomy, comorbidities of the patient, and surgical expertise available. The summaries of open and endovascular repairs of over 210 cases from literature search from 2004 to 2014 are also provided in this review.