Purpose: A systematic review of all patients that have been reported in the literature with abdominal aortic aneurysm (AAA) concomitant with horseshoe kidney (HSK) treated electively by endovascular aneurysm repair (EVAR) is presented. A new grouping system for describing HSK vasculature is implemented. Materials and Methods: We searched for published manuscripts using the Medical Subject Headings terms “abdominal aortic aneurysm,” “AAA,” “EVAR,” “endovascular aneurysm repair,” and “horseshoe kidney” in PubMed, Google Scholar, Scopus, and National Center for Biotechnology Information databases. Inclusion criteria include all published material of patients with AAA with HSK treated electively by an endovascular approach. We excluded patients who were treated by a hybrid or open repair or patients with ruptured AAA. Statistical analysis was carried out using SPSS Statistics version 25 (IBM Corp, Armonk, New York) software. Results: A total of 50 patients from 30 studies were included for analysis. Males made up 88% (n=44) of the population. The median age for this cohort was 70 years (range: 47–86 years). Median aneurysmal diameter was 6.0 cm (range: 4.0–10.3 cm). The median operative time for endovascular repair was 84 minutes (range: 40–332 minutes). The most common graft used was Zenith, used in 40% (n=20) of the cases, followed by Endurant in 14% (n=7). The overall complication rate was 14% (n=7). The median follow-up was 19 months (range: 1–108 months). While comorbidities did not appear to impact outcomes significantly, median operative times for smokers were higher than those in nonsmokers, 84 versus 118 minutes, respectively (p=0.048). Univariate linear regression modeling of aneurysmal size with age, operative time, and length of stay revealed a significant coefficient association between aneurysmal size and operative times. After adjusting for comorbidities and aneurysmal size, prior history of chronic kidney disease significantly increased odds for renal infarction. Conclusion: This review presents the most complete data set possible of patients with concomitant HSK and AAA treated by an endovascular approach. Furthermore, the A + B + C classification for grouping the HSK vasculature is implemented. This systematic review suggests EVAR to be an excellent option with low complication rates for the treatment of AAA in patients with HSK.