Aneurysms of the distal anterior cerebral artery (DACA) are rare, representing between 1% and 9% of all intracranial aneurysms. The best treatment strategy for these aneurysms continues to be debated. We conducted a systematic review of the literature to evaluate the safety and efficacy of treatment strategies of DACA aneurysms. A systematic search of Medline, Embase, Scopus, and Web of Science was performed for studies published from January 2000 to August 2015. We included studies describing treatment of DACA aneurysms with ≥10 patients. Random effects meta-analysis was used to pool the following outcomes: complete occlusion, technical success, periprocedural morbidity/mortality and stroke rates, aneurysm recurrence/rebleed, and long-term neurologic morbidity/mortality. Thirty studies with 1329 DACA aneurysms were included. Complete occlusion was 95% (95% confidence interval [CI], 91.0%-97.0%) in the surgical group and 68% (95% CI, 56.0%-78.0%) in the endovascular group (P < 0.0001). Aneurysm recurrence occurred in 3% (95% CI, 2.0%-4.0%) after surgery and in 19.1% (95% CI, 12.0%-27.0%) after endovascular treatment (P < 0.0001). Overall neurologic morbidity and mortality were 15% (95% CI, 11.0%-21.0%) and 9% (95% CI, 7.0%-11.0%) after surgery and 14% (95% CI, 10.0%-19.0%) (P= 0.725) and 7% (95% CI, 5.0%-10.0%) (P= 0.422) after endovascular treatment, respectively. Overall long-term favorable neurologic outcome was 80% and it was equal in both groups (80%; 95% CI, 73.0%-85.0% in the surgical group and 80%; 95% CI, 72.0%-87.0% in the endovascular group) (P= 0.892). Our meta-analysis showed that both treatment modalities are technically feasible and effective with sufficient long-term aneurysm occlusion and acceptable recurrence/rebleed rates. Surgical treatment is associated with superior angiographic outcomes. There were no substantial differences in procedure-related morbidity and mortality. These findings are important because they suggest that therapy of DACA aneurysms should be performed on a selective, case-by-case basis to maximize patient benefits.