Infra-optic course of the anterior cerebral artery (IOA) is rare and approximately 55 cases of this anomaly have been described. We present a case of a ruptured anterior communicating artery (ACoA) aneurysm arising at the junction between the left IOA and the bilateral A2 segments, at which the right A1 segment was absent. One of the recurrent arteries of Heubner branched off directly from the aneurysmal dome, and was obstructed at aneurysmal neck clipping via an anterior interhemispheric (AIH) approach. In this report, accompanied anatomical variations and surgical approaches for ACoA aneurysms with IOA are reviewed. An IOA is frequently associated with other vascular anomalies, and the origin of functionally important recurrent arteries of Heubner is also variable. Preoperative accurate evaluation of vessel structures and the maximal exposure at surgery are very important. Pterional approach from the ipsilesional side is reportedly to be safe, but interhemispheric approach is also suggested to be effective as to full exposure to recognize the perianeurysmal anatomical structures including potential vessel anomalies.