Kaposi sarcoma (KS) is an acquired immunodeficiency syndrome-defining condition that primarily manifests as mucocutaneous lesions; however, other organs have been implicated in disseminated disease. Fortunately, since the development of antiretroviral therapy, the incidence of KS among patients with human immunodeficiency virus has significantly declined. We report an atypical case of a rapidly progressing pulmonary KS to highlight the importance of prompt recognition of this condition, which can be challenging to distinguish from other pulmonary infectious diseases in immunocompromised individuals, as well as discuss the current treatment for this disease.