Type 4 persistent primitive olfactory artery associated with contralateral accessory middle cerebral artery arising from the fenestrated segment of the distal anterior cerebral artery.

Persistent primitive olfactory artery (PPOA) is a rare variation of the proximal anterior cerebral artery (ACA) that generally follows an extreme anteroinferior course and takes a hairpin turn before continuing to the A2 segment of the ACA (type 1). There are four other types of extremely rare variations. The type 4 variation continues to the accessory middle cerebral artery (MCA) instead of the ACA. Only a few cases have been reported. We herein report a case of type 4 PPOA in which the contralateral side of the accessory MCA arose from the fenestrated segment of the distal ACA. No similar cases were found in the relevant English-language literature. For the identification of these variations on MR angiography, volume-rendering images were superior to maximum-intensity-projection images.