Congenital lobar emphysema (CLE) is a developmental anomaly of the lower respiratory tract characterized by hyperinflation of one or more pulmonary lobes in the absence of extrinsic bronchial obstruction. We present a case of a 24-year-old male, nonsmoker who presented with shortness of breath and severe left sided chest pain. A chest x-ray was significant for a very large left-sided pneumothorax and chest CT showed lobar emphysematous changes. Video assisted thoracoscopic surgery (VATS) and lobectomy was subsequently performed after persistence of pneumothorax despite chest tube insertion and conservative management. Surgical pathology of resected specimen showed chronic emphysematous changes with patchy chronic organizing pneumonitis. Histology showed advanced emphysematous changes of pulmonary parenchyma consistent with congenital lobar emphysema. This finding combined with features seen on computed tomography of the chest led to the diagnosis of congenital lobar emphysema. This case demonstrated that CLE can be a cause of tension pneumothorax in adults in rare cases.