Prolonged hypoxemic respiratory failure and evidence of lung organization and fibrosis are features of an ARDS subgroup that is variably identified as "late," "persistent," or "fibroproliferative" ARDS. Early reports suggested that patients with late ARDS had a high mortality unless treated with corticosteroids. A large recent study with improved methodology has demonstrated that despite improvements of pulmonary physiology, corticosteroids do not change mortality of patients who continue to meet ARDS criteria 7 to 28 days after onset of acute lung injury. Additionally, there is no compelling evidence that persistent ARDS confers a higher mortality than that of ALI/ARDS. Observational and interventional studies are needed to increase understanding of the incidence, best management, and outcomes of patients with persistent ARDS.