An infraoptic course of the anterior cerebral artery (ACA) is a rare anomaly of the anterior part of the circle of Willis. About 56 cases have been reported, often in association with cerebral aneurysm. We describe a case involving an infraoptic ACA in which a ruptured middle cerebral artery aneurysm was also present. A 52-year-old man experienced a sudden onset of severe headache without focal neurological deficits. Computed tomography demonstrated diffuse subarachnoid hemorrhage. Three-dimensional computed tomographic angiography and conventional angiography revealed a saccular aneurysm in the left middle cerebral artery. An anomalous arterial branch, originating from the right internal carotid artery at the level of the ophthalmic artery was also visible. This vessel followed an infraoptic course. The aneurysm was successfully embolized with coils. We review the literature for the possible genesis of this anomaly and consider treatment of the associated aneurysm.