Aneurysms located at the A1 segment of the anterior cerebral artery are considered rare and unique entities. Endovascular treatment (EVT) is effective in preventing aneurysmal bleeding. However, EVT for A1 aneurysms is difficult due to their distinctive configurations. A current review of EVT for A1 aneurysms is lacking. Therefore, we focused on the available literature on this specific issue. To more clearly expound this entity, we also provided some illustrative cases. The A1 segment can be equally divided into the proximal, middle, and distal segments. Proximal aneurysms are most common and difficult to treat via EVT. The A1 segment has a complex anatomy and many important branches. Due to the small size, predominant posterior direction, and sharp upturn of the microcatheter from the parent artery, microcatheter positioning and support is difficult for A1 aneurysms. EVT for A1 aneurysms mainly includes reconstructive and deconstructive strategies. The complications of EVT for A1 aneurysms include aneurysmal perforation, thromboembolic events, and coil protrusion related to stent-assisted embolization. A1 aneurysms represent rare and difficult vascular lesions. EVT is quite challenging for A1 aneurysms due to their distinctive configurations. The outcomes are acceptable.