A 59-year-old woman presented with subarachnoid hemorrhage. Conventional angiography and three-dimensional computed tomography (3-D CT) angiography showed a saccular aneurysm at the junction of the azygos anterior cerebral artery (ACA) and the left A₁ segment. This aneurysm was associated with a fenestration of the right hypoplastic A₁ segment. The aneurysm neck was completely clipped, preserving the azygos ACA and other perforators. Aneurysm of the azygos ACA is almost always located at the distal bifurcation, and rarely at the proximal end. Proximal azygos ACA aneurysm can mimic anterior communicating artery aneurysm. Therefore, accurate preoperative diagnosis is critical using 3-D CT angiography as well as conventional angiography, and close follow up of patient is necessary to monitor for development of a de novo aneurysm at the distal bifurcation of the azygos ACA.