As the prevalence of tuberculosis (TB) declines in the developed world, the proportion of mycobacterial lung disease due to nontuberculous mycobacteria (NTM) is increasing. It is not clear whether there is a real increase in prevalence or whether NTM disease is being recognized more often because of the introduction of more sensitive laboratory techniques, and that more specimens are being submitted for mycobacterial staining and culture as the result of a greater understanding of the role of NTM in conditions such as cystic fibrosis, posttransplantation and other forms of iatrogenic immunosuppression, immune reconstitution inflammatory syndrome, fibronodular bronchiectasis, and hypersensitivity pneumonitis. The introduction of BACTEC liquid culture systems (BD; Franklin Lakes, NJ) and the development of nucleic acid amplification and DNA probes allow more rapid diagnosis of mycobacterial disease and the quicker differentiation of NTM from TB isolates. High-performance liquid chromatography, polymerase chain reaction, and restriction fragment length polymorphism analysis have helped to identify new NTM species. Although treatment regimens that include the newer macrolides are more effective than the earlier regimens, failure rates are still too high and relapse may occur after apparently successful therapy. Moreover, treatment regimens are difficult to adhere to because of their long duration, adverse effects, and interactions with the other medications that these patients require. The purpose of this article is to review the common presentations of NTM lung disease, the conditions associated with NTM lung disease, and the clinical features and treatment of the NTM that most commonly cause lung disease.