The azygos anterior cerebral artery (ACA) is a rare anatomical anomaly. Clipping surgery has been conducted in approximately 30 reported cases because it is frequently associated with aneurysms. However, few cases in which coil embolization was performed have been reported. We report three cases of coil embolization for distal ACA aneurysms with distal azygos ACA at our institution in 7 years. All patients were over 65-year-old women with saccular aneurysms larger than 7 mm; two with subarachnoid hemorrhage and one with an unruptured aneurysm. No patient had surgical complications associated with coil embolization. Coil embolization is also useful for large aneurysms in the distal azygos ACA, and its indication for treatment could be broadened.