The aneurysm arising from fenestrated proximal anterior cerebral artery (ACA) is considered to be unique. The authors report a case of a 59-year-old woman who presented with a subarachnoid hemorrhage (SAH) secondary to a ruptured aneurysm originating from the fenestrated A1 segment of right ACA. The patient had another unruptured aneurysm which was located at the right middle cerebral artery bifurcation. She was successfully treated with surgical clipping for both aneurysms. From the previously existing literatures, we found 18 more cases (1983-2011) of aneurysms associated with fenestrated A1 segment. All cases represented saccular type of aneurysms, and 79% of the patients had SAH. There were three subtypes of the fenestrated A1 aneurysms depending on the anatomical location, relative to the fenestrated segment. The most common type was the aneurysms located on the proximal end of fenestrated artery (82%). Azygos ACA and hypoplastic A1 were frequently accompanied by the aneurysm (33% and 31%, respectively), and multiple aneurysms were shown in three cases (16%). Considering that fenestrated A1 segment is likely to develop an aneurysm, which has high risk of rupture, early management may benefit patients with aneurysms accompanied by fenestrated proximal ACA.