Bronchiolitis obliterans organizing pneumonia (BOOP) is a disease of the small airways characterized by intraluminal polyps of myxoid connective tissue. Although various infectious and non-infectious agents have been implicated as possible precipitants of BOOP, the concomitant occurrence of BOOP with human immunodeficiency virus (HIV) infection has rarely been described. We describe a unique case in which BOOP was a presenting feature in a patient with newly diagnosed AIDS, and we review the literature of BOOP occurring in the setting of HIV infection.