BACKGROUND: Pulmonary lymphangioleiomyomatosis is a rare disease that occurs 
mainly in women of reproductive age. The clinical characteristics include 
recurrent spontaneous pneumothorax and progressive dyspnea. The features of 
chest computed tomography are nearly pathognomonic with the detection of 
bilateral thin-walled cysts.
CASE REPORT: A 32-year-old female presented with sudden-onset right-sided chest 
pain, which aggravated during inspiration, dyspnea at exertion, and cough. She 
had a history of bilateral recurrent spontaneous pneumothorax. Physical 
examination showed reduced pulmonary sounds on the right side. Chest X-ray 
confirmed the diagnosis of recurrent right-sided pneumothorax. Computed 
tomography showed multiple bilateral bullae. Video-assisted pleurectomy, bulla 
resection, and bulla coagulation were performed. The diagnosis of pulmonary 
lymphangioleiomyomatosis was confirmed by pulmonary biopsy. After pleurectomy, 
the patient remained symptom-free without recurrent pneumothorax.
CONCLUSION: Pulmonary lymphangioleiomyomatosis is a rare cause of recurrent 
pneumothorax and should be considered a differential diagnosis, especially in 
young women with diffuse bilateral bullous emphysema or tuberous sclerosis.