Arteriovenous malformation (AVM) fed by the artery of Percheron (AOP) is extremely rare, with only one case described in the literature to date. We herein present a case of a 43-year-old female with hemorrhagic thalamic AVM mainly supplied by the AOP, who subsequently underwent conservative management and recovered well except for mild right hemiparesis. This case highlights that the AOP is a rarely recognized but clinically significant entity and should be identified when dealing with AOP-associated neurovascular disorder as described.