Staphylococcus aureus: is a common bacterial organism infecting children with cystic fibrosis (CF). Emerging evidence suggests early lower airway infection with this organism in young children with CF results in the deterioration of lung function, poorer nutrition parameters and heightens the airway inflammatory response. Despite contributing significantly to the burden of early lung disease among this group, there are ongoing controversies in the management of S. aureus infection, and gaps in our understanding of exactly how this organism causes lung disease. To reduce the morbidity and mortality of early infection ongoing research is needed to: (i) understand the early host immune response that enables this pathogen to reside within the CF lung; (ii) determine if there are organism specific factors that are associated with CF lung disease; and (iii) clarify the utility of anti‐staphylococcal antibiotic prophylaxis and/or eradication in the treatment of this patient population. Pediatr Pulmonol. 2013; 48:1151–1159. © 2013 Wiley Periodicals, Inc.