BACKGROUND: Extracranial carotid artery aneurysms (ECAAs) are extraordinarily rare, representing approximately 0.4%-4% of all extracranial artery aneurysms. As medical technology has advanced, new approaches for ECAAs treatment can be performed. Nevertheless, there is currently no consensus on the best therapeutic approach due to the information scarcity. METHODS: We performed a systematic review of all published ECAA cases in Scopus, Medline, Web of Science, and Google Scholar to retrieve all available studies up to March 2024. RESULTS: Eighty-eight studies reporting on a total of 359 patients presenting ECAAs were included. The mean age at diagnosis was 53 years. Most patients were male (58.4%). The primary presenting symptoms were pulsatile mass (31.2%), ischemia (24.7%), pain (9.75%), and dizziness (8.36%). Overall, the leading etiologies of aneurysms were atherosclerosis (34.2%), trauma (10%), and vasculitis (5.57%). Surgery was performed in 68.5% of patients, 26.7% underwent endovascular procedures, and 3.9% received conservative management. CONCLUSIONS: ECAAs are a rare clinical condition. However, a great percentage of patients could present with ischemic symptoms. Similarly, cardiovascular risk factors present as the most prevalent comorbid conditions associated with these vascular aberrancies. With this systematic review, we seek to provide insight into extracranial carotid aneurysms, identifying areas of opportunity in both the diagnosis and management of this pathology and the standardization of clinical reporting and case classification. These findings underscore the need for future research to improve the understanding and approach to this complex clinical condition.