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Background: Sarcoidosis is a multisystem inflammatory disorder characterized by non-caseating granulomas with predominantly lung manifestations that can cause restrictive or, less commonly, obstructive lung disease. Rarely is pneumothorax a manifestation of sarcoidosis; it has been reported as an early finding in sarcoidosis, but it is typically attributed to ruptured bullae, cysts, or pleural granulomas. Case Presentation: We present a unique case of spontaneous pneumothorax attributed to biopsy-proven sarcoid disease in a patient with no prior history of pulmonary complications. The patient’s rapid development of novel pulmonary sequelae necessitates treatment of sarcoidosis early in the disease course. Conclusion: We recommend a treatment plan of appropriate chest tube placement in the affected lung(s) and prompt steroid therapy for patients with sarcoidosis presenting with a pneumothorax. We also review the literature for the etiology, pathophysiology, and presentation of pneumothorax in sarcoidosis, treatment of this sequela, and pulmonary function test findings in these patients. [ABSTRACT FROM AUTHOR]