Pleural fibrosis can result from a variety of inflammatory processes. The response of the pleural mesothelial cell to injury and the ability to maintain its integrity are crucial in determining whether normal healing or pleural fibrosis occurs. The pleural mesothelial cell, various cytokines, and disordered fibrin turnover are involved in the pathogenesis of pleural fibrosis. The roles of these mediators in producing pleural fibrosis are examined. This article reviews the most common clinical conditions associated with the development of pleural fibrosis. Fibrothorax and trapped lung are two unique and uncommon consequences of pleural fibrosis. The management of pleural fibrosis, including fibrothorax and trapped lung, is discussed.