Arteriopathy associated with human immunodeficiency virus infection and clinical acquired immunodeficiency syndrome is well-documented. The pathophysiology of this arteriopathy may vary in different vascular beds. Although arteriopathy of central nervous system (CNS) circulation has been recognized in pediatric patients since the late 1980s, there are no reported cases of CNS arteriopathy in adults. We present the first reported case of adult CNS arteriopathy in a human immunodeficiency virus-positive patient who succumbed to complications secondary to diffuse aneurysmal disease of the Circle of Willis.