During the past 13 yrs it has become evident that pulmonary complications occur frequently and are often life-threatening in patients infected with the human immunodeficiency virus (HIV). Moreover, the spectrum of HIV-related pulmonary diseases has enlarged considerably and it now includes numerous infectious and non-infectious conditions. Because clinical and radiological presentation is generally nonspecific and often altered by diminished signs of inflammation or prophylactic measures, and multiple simultaneous complications frequently occur, an early aetiological diagnosis is essential. The diagnostic work-up of these patients must be straightforward. Examination of (induced) sputum and bronchoalveolar lavage fluid are the most important procedures. If they are not diagnostic, transbronchial biopsy, transbronchial needle aspiration, computed tomography and echocardiography are most often revealing. However, we must be continuously prepared to face new complications in the course of this devastating disease.