Cerebral aneurysms arising from fenestration of the A1 portion of the anterior cerebral artery (ACA) (A1 fenestration) with the accessory middle cerebral artery (MCA) is rare. Herein, we report a ruptured cerebral aneurysm arising from A1 fenestration combined with the accessory MCA that was successfully treated with coil embolization. A 51-year-old woman suddenly experienced a severe occipital headache and was admitted to our hospital. Detailed examination revealed subarachnoid hemorrhage due to a cerebral aneurysm arising from A1 fenestration combined with the accessory MCA. Hence, coil embolization was performed, and a favorable outcome was obtained. Coil embolization of the cerebral aneurysm arising from the A1 fenestration of the ACA combined with the accessory MCA is considered to be useful.