Pulmonary complications of HIV infection increase as the disease progresses. Although the most common cause of pulmonary disease continues to be infection, a number of noninfectious entities should be considered when evaluating the HIV-infected individual with pulmonary symptoms. With the advent of highly active antiretroviral therapy, there have been significant changes in the epidemiology of these diseases. Improved understanding of pathogenesis has led to new therapies, especially for HIV-associated pulmonary hypertension. In those patients in whom a thorough microbiologic survey reveals no infection, one should consider noninfectious entities; these can be broadly categorized into 3 groups: malignant, inflammatory, or pulmonary vascular disease. Clinical history is critical in providing clues, but bronchoscopic visualization and tissue sampling are often required to confirm many of these diagnoses. As successful antiretroviral therapy continues to extend the lives of HIV-infected patients, the medical community should be able to recognize and diagnose these noninfectious pulmonary diseases.