The azygos anterior cerebral artery is a rare variant, characterized by the absence of the anterior communicating artery and the union of two proximal segments of the anterior cerebral artery, forming a single trunk and ascending through the interhemispheric fissure. The incidence in the population varies from 0.3 to 2%. The presence of occlusion for this vessel causes bifrontal infarcts, with potentially devastating functional consequences, hence the importance of recognizing this anatomical variation in imaging exams.