A case of mediastinal fibrosis with occlusion of the right inferior and middle lobe pulmonary veins is described. The patient had severe hemoptysis, and the diagnosis was established with thoracotomy. Review of the literature emphasizes the lethal nature of this syndrome if untreated. Resection of the involved bronchopulmonary tissue, when feasible, offers good palliation, although the long-term prognosis is unpredictable.