Understanding mechanisms that underlie lung disorders is crucial to achieving optimum care and improved outcomes in pulmonary medicine. Extensive investigations have revealed that inflammation displays an active role in the pathogenesis of these diseases. The byproduct of these inflammatory reactions has been shown to propagate pulmonary disease in consonance with alteration in haemostatic balance. It is now apparent that the two phenomena constitute an interwoven relationship with protective but damaging effects, when dysregulated. However, the precise role of coagulation abnormalities in pulmonary pathology is still evolving. A large body of evidence suggests that an imbalance in intra-alveolar procoagulant and fibrinolytic activities occurs in a variety of lung conditions. This imbalance may even herald a number of pulmonary diseases. Its sequelae have been observed in lung parenchyma of humans and in animal models of lung inflammation. As the pathogenesis of coagulation-related lung diseases continues to be unraveled, therapeutic measures to mitigate pulmonary disease-specific coagulopathy are emerging. Current efforts are directed at depicting multifaceted molecules capable of selective but simultaneous interference with relevant aspects of the dual coagulation-fibrinolytic pathway.