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Birt-Hogg-Dubé (BHD) syndrome is an autosomal and predominantly inherited disorder. We report 3 cases of BHD syndrome. Case 1A 24-year-old man was admitted to our hospital due to left-sided spontaneous pneumothorax. He had a previous drainage history of right-sided spontaneous pneumothorax. In operation, we resected the cyst at segment 8 of the left lung and covered the lung using polyglycolic acid( PGA) sheet. Case 2A 47-year-old man was admitted to our hospital due to right-sided spontaneous pneumothorax. He had a previous surgical histor