Objectives: The purpose of this paper is to report the different modalities for the treatment of isolated internal iliac artery aneurysms (IIIAA), as well as their outcomes. Methods: We performed a systematic review of the literature (database searched: PubMed, Web of Science, Scopus, Cochrane Library; last search: April 2020). We included articles reporting on the outcomes for IIIAA interventions comprising at least 5 patients. Studies were included when presenting extractable outcome data regarding intraoperative and/or early results. We performed meta-analyses of proportions for different outcomes, using random effects model. Results: Thirteen non-randomized studies were included (192 patients with 202 IIIAA). IIIAA were symptomatic in the 18.1% (95%CI 9.3-26.9; I2 54.46%, P = .019). Estimated mean IIIAA diameter was 46.28 mm (95%CI 39.72-52.85; I2 88.85%, P < .001). Open repair was performed in 21/202 cases. Endovascular treatments were: embolization (81/181), embolization and hypogastric artery coverage (79/181), hypogastric artery coverage by stent-grafting (15/181), stent-grafting in the hypogastric artery (6/181). Overall estimated technical success (TS) rate was 91.6% (95% CI 86.8-95.5; I2 45.82%, P = .031). TS rate was 94.5% for open surgery (95%CI 85.3-100; I2 0%, P = .907), and 89.7% for endovascular repair (95%CI 83.8-95.6; I2 55.43%, P = .006). Estimated overall 30-day mortality was 3.1% (95%CI 0.8-5.4; I2 0%, P = .969). Mortality rates after open surgery and endovascular repair were 8.2% (95%CI 3.4-19.8; I2 0%, P = .545) and 2.8% (95%CI 0.5-5.1; I2 0%, P = .994), respectively. Estimated mean follow-up was 32.63 months (95%CI 21.74-43.53; I2 94.45%, P < .001). During this timeframe, IIIAA exclusion was preserved in 92.8% of the patients (95%CI 89.3-96.2; I2 0%, P = .797). Buttock claudication occurred in 13.9% of the patients (95%CI 8.7-19.2; I2 0%, P = .622). Conclusions: IIIAA are frequently large, and symptomatic at presentation. Several treatments are proposed in literature, open and endovascular, both with good results. The endovascular treatment is the preferred method of treatment in literature, since it offers good short- to mid-term results and low early mortality. Buttock claudication after hypogastric artery exclusion is a common complication.