Imaging of the cerebral arterial circulation is key in neurological diseases and knowledge of anatomical variants is very important in both radiologic interpretation and interventional procedures. We present a case of A1 segment hypoplasia and azygos A2 anterior cerebral artery anomalies with the aim of discussing the normal anatomy, embryological perspectives and the most common variants of anterior cerebral artery.

KEY WORDS: Anterior cerebral artery; azygos; hypoplasia,