Although Burkholderia cepacia colonizes a relatively small proportion of individuals with cystic fibrosis (CF), it is associated with significant morbidity and mortality, and has had a profound impact on infection control practices. This article reviews the current understanding of the epidemiology of B. cepacia infection, describes important recent developments in the microbiology and taxonomy of this species, and presents issues that remain obstacles to defining the optimal management of B. cepacia infection in CF.