The conjunction of a glioma with an arteriovenous malformation (AVM) is exceptionally rare. We report the case of a malignant pleomorphic xanthoastrocytoma located on the vicinity of an untreated AVM that was removed without interference with the AVM. The question posed by such concurrent lesions is which to manage first. It appears more logical to treat the tumour first because of the elevated incidence of associated high-grade gliomas.