Fibrothorax is a severe form of pleural fibrosis that causes the fusion of the visceral and parietal pleura, thereby eliminating the pleural space. It occurs following an inflammatory process triggered by infection, systemic diseases, direct pleural injury, certain drugs, or environmental exposures. Undrained pleural fluid can also stimulate an inflammatory cascade, leading to the development of fibrothorax. This may result in the contracture of the affected lung, reducing lung volumes and leading to extrapulmonary restrictive lung disease. Clinically, patients experience dyspnea, occasional chest pain, and may develop hypoxia, hypercarbia, pulmonary hypertension, and cor pulmonale. Radiologic imaging is often diagnostic, demonstrating uniform peripheral linear densities on chest x-ray. CT of the chest is best for diagnosis and can demonstrate pleural thickening with soft-tissue attenuation, reduced thoracic volume, and occasional calcifications. Prevention of the development of a fibrothorax relies on prompt drainage of retained pleural fluid. In cases where tube thoracostomy fails, decortication via VATS or thoracotomy may be indicated to restore normal lung function.