Objective The aim of this study was to report the clinical presentation and treatment outcomes of patients treated for IAAD with and without abdominal aortic aneurysm (AAA) in a single academic institution in South America.Materials and Methods A retrospective review of all patients with IAAD with or without concomitant AAA between January 2002 and December 2023 from a single academic hospital was performed.Results Eighteen patients with IAAD were diagnosed of whom 13 (72.2%) were males. Median age was 63 years (range: 43-88 years). Sixteen (88.8%) patients presented with symptoms, and in two (11.1%) asymptomatic patients IAAD was an incidental finding. Ten (55.5%) patients had concomitant abdominal aortic aneurysm (AAA), with a median size of the aneurysm of 49.5 mm (range: 44-66 mm). No statistical differences in baseline characteristics were seen between patients with concomitant IAAD and AAA and patients with only IAAD. Seven (38.8%) patients presented chronic dissection, and 11 (61.1%) patients had acute dissection. Five (27.7%) patients were treated conservatively with blood pressure, pain control, and antiplatelets; endovascular surgery was performed in eight (44.4%) patients and open surgery in five (27.7%) patients. The complication rate was 22.2% (n = 4), and the mortality rate was 0%. Median follow-up was 36 months (range: 6-240 months). Complete remodeling was seen in all patients except two patients who underwent conservative treatment. Of those, one had partial remodeling, and the other no changed.Conclusion Isolated aortic dissection of the abdominal aorta is an uncommon condition, with acceptable different treatment strategies, from conservative to invasive treatments. Sometimes IAAD can concur with AAA, and when so, invasive treatment might be considered. More studies describing the natural history of AAA and its association with IAAD are warranted, as well as further research reporting long-term outcomes on aortic remodeling after different treatment modalities.