Skin and/or pulmonary involvement occur frequently in Langerhans cell histiocytosis (LCH) whereas nail involvement is rare. Herein, we present a case of LCH with initial nail disease and subsequent lung involvement causing recurrent pneumothoraces. Systemic chemotherapy was applied and intrapleural bleomycine and thoracoscopic pleurodesis with talc were performed. Although prognosis is not satisfactory in LCH with recurrent pneumothorax and nail involvement, the patient is under follow-up with no evidence of skin lesions, nail involvement, and pneumothorax for the last 10 months.