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Pulmonary lymphangioleiomyomatosis (LAM) is a disease, which is most commonly seen in women of childbearing age. The objective of this article was to provide education about the typical clinical presentation, radiologic findings, histology, treatment approaches, and differential diagnosis. Pulmonary LAM is a cystic lung disease, usually generalized and progressive and extremely difficult to treat and is considered to have a poor prognosis. Patients with LAM often present with an insidious onset of dyspnea; this could be secondary to pneumothorax. However, it could also be present as chylothorax and hemoptysis. We discussed a case who presented with chest pain and shortness of breath due to pneumothorax and retrospectively diagnosed with LAM.