Human brucellosis is a disease of protean manifestations, and has been implicated in complications and focal disease in many human organ systems. However, little is collectively known about the background, the course, the clinical characteristics, the diagnostic issues raised, and the short- and long-term therapeutic approaches in patients with aortic involvement as a complication of brucellosis. With the aim to glean from the literature useful information to better understand and manage this complication, a computerized search without language restriction was conducted using PubMed and SCOPUS. An article was considered eligible for inclusion in the systematic review if it reported data on patients with involvement of the aorta due to a Brucella infection. The epidemiologic and clinical characteristics of 44 cases of brucellar aortic involvement found through the systematic review of the literature were analyzed together with those of two new cases that we treated in the recent past. This complication involved the ascending thoracic aorta in 18 cases (in 16 of them as a consequence of brucellar endocarditis), and the descending thoracic aorta or the abdominal aorta in the remaining 30 cases. In the latter it was associated with spondylodiscitis of the lumbar spine in 13 cases. History of or symptoms indicative of brucellosis were not universally present. Brucellar aortic involvement represents a possibly underdiagnosed and underreported complication with major morbidity and mortality potential. Experience with novel invasive therapeutic approaches remains limited. Early suspicion through detailed history and diagnosis, aided by advances in aortic imaging, would allow for better planning of therapeutic interventions.