Artery of Percheron (AOP) as main feeder artery of arteriovenous malformation (AVM) is extremely rare. Two cases of thalamic AVM fed by AOP have been reported to date and only one AVM been removed by microsurgery when attempt of intervention embolization failed. Midbrain AVM fed by AOP has not been reported yet. Here, we presented the first successful embolized case of midbrain AVM supplied by the AOP in a 10-year-old boy, who suffered dual oculomotor nerve palsy and secondary hemorrhage before embolization. During endovascular embolization, selective angiography by 1.2 Fr. Magic microcatheter showed an intranidal aneurysm located on the distal AOP. Two injections of a 1:4 ratio mixture of NBCA-MS completely occlude the nidus and intranidal aneurysm with no complications occurred. The child recovered well and the oculomotor deficits improved. This case highlighted that AOP is a clinically significant branch associated with AVM in midbrain and thalamus. Moreover, intervention embolization of midbrain AVM fed by AOP is a considerable therapeutic strategy.