Introduction: Carotid cavernous fistulas (CCFs) are rare, usually follow head trauma or aneurysmal rupture. Recent treatment options include endovascular techniques such as flow diversion devices (FDDs). Objective: To present our case treated with FDD application with transarterial cavernous-sinus coiling and present a systematic review on the use and effectiveness of FDDs in CCF treatment. Materials and methods: We present our case of CCF treatment with FDD. A search was also conducted in PubMed, EMBASE and Cochrane until November 2020. Reference lists were also cross-checked. Results: Including our case, thirty-eight patients were identified with a CCF that was treated with FDDs in sixteen studies. Twenty-two patients were females, nine were males and the rest unidentified. The mean age was 52,6 years (range 17–86, SD± 19.28). Thirty-six patients suffered from direct and two from indirect CCFs. Single FDD was used in four cases, single FDD with embolic materials in eleven cases, multiple overlapping FDDs were used in six cases and multiple overlapping FDDs with embolic materials were used in seventeen cases. Thirty-five patients (92,1%) had clinical improvement, immediate angiographic occlusion was seen in 44,7% of the cases, while long-term occlusion rate was 100% but with variable follow-up periods. One patient (2,6%) presented with a neurological deficit related to FDD deployment. Conclusion: Targeted treatment of CCFs with single or overlapping FDDs with or without adjunct embolic agents offers a high success rate, both clinically and long-term angiographically compared to other endovascular methods alone. However, further research with multi-center prospective trials is warranted.