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Objective: A case of a carotid-cavernous fistula (CCF) that was close to the superior ophthalmic vein (SOV) that occurred during endovascular catheter navigation and was treated by transarterial coil embolization is reported.Case Presentations: An arteriovenous fistula and eye swelling occurred during percutaneous transluminal angioplasty (PTA) balloon catheter navigation. Suspecting and searching for a carotid-cavernous fistula, the microcatheter passed from the internal carotid artery (ICA) to the SOV. The fistula was considered to have occurred in the cavernous sinus close to the SOV. The fistula was closed by coil embolization from another microcatheter.Conclusion: It is important to be aware that a CCF may occur at the anterior genu of a cavernous ICA during catheter navigation. In the case of a fistula caused by a guidewire/microcatheter, transarterial coil embolization is possible and may be tried first.