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Background: Neurofibromatosis type1 (NF-1) is a hereditary autosomal dominant disease that is accompanied by complications, such as benign and malignant tumors and vascular involvement, including pulmonary hypertension, artery stenosis, and pulmonary artery aneurysm. Spontaneous hemothorax is a rare and lethal complication of NF-1 due to vasculopathy as stenosis or aneurysmal modifications of large intrathoracic vessels, and dysplastic alterations of small vessels in highly vascularized mesenchymal tumors. Most commonly, tumor-related hemothorax is due to neurofibroma, and the most frequently involved artery is the intercostal artery.
Case Presentation: A 48-year-old male referred to our hospital with a chief complaint of dyspnea from a week ago. His symptoms began with pleuritic chest pain followed by dyspnea. The physical examination showed many neurofibromatosis lesions and café-au-lait macules on the skin. Thoracotomy was performed during the hemothorax examination. Moreover, inflammation of the pleura and fibrothorax were reported without any site of bleeding.
Conclusion: Vascular complications should be regarded in NF-1. Any patient presents with hemodynamic disorder or pleural effusion should be examined and treated promptly

Keywords: Hemothorax, Neurofibromatosis Type 1

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