The anterior cerebral artery (ACA) contains anatomical variants that are closely related to its embryology and development. In this study, the authors reviewed the most commonly encountered variants of the ACA and anterior communicating artery. They also reviewed the embryological origins of these variants as well as a variety of associated pathologies. Several variants are described and highlighted with illustrations including: (1) the aberrant origin of the ACA from the internal carotid artery and its developmental association with the ophthalmic artery; (2) the persistent olfactory artery; (3) the azygous ACA; (4) the triplicated ACA; and (5) multiple anterior communicating arteries. The formation of aneurysms is associated with such variants, thus their knowledge and the embryology behind their development are crucial to prevent injury to the patient.