The authors report a unique case of unilateral Moyamoya disease with a rare combination of arteriovenous malformation (AVM) who presented with intracerebral hemorrhage (ICH). A 50-year-old man suffered from sudden onset of mental deterioration and right hemiparesis. Brain computed tomography (CT) showed intracerebral hemorrhage on left thalamus. Brain CT angiography and cerebral digital subtraction angiography (DSA) revealed AVM combined with unilateral moyamoya disease involving left middle cerebral artery (MCA) and choroid plexus in left lateral ventricle. Intraventricular hemorrhage and hydrocephalus were managed conservatively. A rare case of unilateral Moyamoya disease accompanied by a cerebral arteriovenous malformation is described and discussed with review of pertinent literature.