Proximal or A1 segment anterior cerebral artery (ACA) aneurysms are rare aneurysms. They have certain unique properties when compared to all other intracranial aneurysms. They are smaller and prone to an early rupture. Thus, they need an aggressive intervention even when they are less than 7 mm in size. The technical difficulties that arise in their management are based on their location along the length and circumference of the ACA. Accordingly, they have been variously classified in literature. Their intimate relationship to the medial lenticulostriate perforators and the recurrent artery of Heubner is the prime factor in determining the surgical or endovascular strategy and outcome. These aneurysms are commonly associated with vascular anomalies, needing a thorough interpretation of preoperative angiograms. Though there are many series reporting the clinical features and management outcome of proximal ACA aneurysms, all are limited by a small sample size due to the rarity of the pathology. In this article, the pertinent literature related to the surgical nuances associated with dealing with A1 segment aneurysms is reviewed. The clinical presentation, associated anatomical characteristics, microsurgical management, role of endovascular therapy, and outcome are discussed.