Burkholderia cepacia has emerged as an important pathogen in patients with cystic fibrosis (CF) undergoing lung transplantation. Taxonomic analyses have divided B. cepacia into 7 closely related species called genomovars. The prevalence of B. cepacia infection ranges from 2% to 13% in CF patients with genomovar III being most prevalent. Risk factors for the acquisition of B. cepacia include greater severity of underlying CF, increasing age, having a sibling colonized with B. cepacia, and previous hospitalizations. One-year survival in patients with CF undergoing lung transplantation usually have been reported to be less than 70%. The antimicrobial resistance pattern of B. cepacia has correlated with inconsistent effect on survival. Presence of genomovar III also has been linked with survival of B. cepacia-infected patients; the survival rates were inferior for genomovar III as compared with non-genomovar III patients. Standard infection control techniques including isolation of both colonized and noncolonized individuals with frequent sputum culture have been recommended to prevent CF patients from acquiring B. cepacia infection. B. cepacia is inherently resistant to antipseudomonal antibiotics, colistin, and polymyxin. The combination of 2 or more antibiotics usually is recommended for the treatment of B. cepacia infections. Studies delineating the epidemiology and influence of B. cepacia infection on lung transplantation are warranted. This is a US government work. There are no restrictions on its use.