Tehran University of Medical Sciences Acta Medica Iranica 0044-6025 46 3 2008 06 15 269 282 M. Vaziri A. Pazooki L. Zahedi 2015 09 28 Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax. https://acta.tums.ac.ir/index.php/acta/article/view/3480 https://acta.tums.ac.ir/index.php/acta/article/download/3480/3457