Background: A high incidence of anatomical variations in the origin of the branches of the aortic arch has been reported, Nowadays, this variation is considered the most frequent in the aortic arch, its prevalence being estimated between 0.5% and 2.5% of the population. To understand its origin, knowledge of embryonic development is necessary. Methods: We searched the MEDLINE, Scopus, Web of Science, Google Scholar, Cumulative Index to Nursing and Allied Health Literature, and Latin-American literature and caribean of health sciences databases with dates ranging from their inception to June 2023. Study selection, data extraction, and methodological quality were assessed with the guaranteed tool for anatomical studies (Anatomical Quality Assurance). Finally, the pooled prevalence was estimated using a random effects model. Results: Thirty-nine studies were found that met the eligibility criteria. Twenty studies with a total of 41,178 subjects were included in the analysis. The overall prevalence of an ARSA variant was 1% (95% confidence interval = 1%–2%), the clinical findings found are that if ARSA is symptomatic it could produce changes in the hemodynamic function of the thoracocervical region in addition to other associated symptomatic complications in surrounding structures. Conclusions: ARSA can cause several types of alterations in the cervical or thoracic region, resulting in various clinical complications, such as lusory dysphagia. Hence, knowing this variant is extremely important for surgeons, especially those who treat the cervico-thoracic region. The low prevalence of ARSA means that many professionals are completely unaware of its existence and possible course and origin. Therefore, this study provides detailed knowledge of ARSA so that professionals can make better diagnoses and treatment of ARSA.