The anterior cerebral artery (ACA) varies considerably and this complicates the description of the normal anatomy. The segmentation of the ACA is mostly agreed on by different authors, although the relationship of the pericallosal and callosomarginal arteries (CmA) is not agreed upon. The two basic configurations of the ACA are determined by the presence or absence of the CmA. The diameter, length and origin of the cortical branches have been measured and described by various authors and display great variability. Common anomalies of the ACA include the azygos, bihemispheric, and median anterior cerebral arteries. A pilot study was done on 19 hemispheres to assess the variation of the branches of the ACA. The most common variations included absence and duplication. The inferior internal parietal artery and the CmA were most commonly absent and the paracentral lobule artery was the most frequently duplicated (36.8%). The inferior internal parietal artery originated from the posterior cerebral artery in 40.0% and this was the most unusual origin observed. It is important to be aware of the possibility of variations since these variations can have serious clinical implications. The knowledge of these variations can be helpful to clinicians and neurosurgeons. The aim of this article is to review the anatomy and variations of the anterior cerebral artery, as described in the literature. This was also compared to the results from a pilot study.