{Reference Type}: Case Reports {Title}: An elderly woman with Birt-Hogg-Dubé syndrome having multiple pulmonary cysts mimicking lymphangioleiomyomatosis. {Author}: Sekimoto Y;Yamawaki I;Iwabuchi C;Nishino K;Takahashi K;Seyama K; {Journal}: Respir Investig {Volume}: 62 {Issue}: 3 {Year}: 2024 May 28 暂无{DOI}: 10.1016/j.resinv.2024.03.005 {Abstract}: The characteristics of the pulmonary cysts on the high-resolution computed tomography (HRCT) chest images are an important diagnostic clue to distinguish among cystic lung diseases. The diagnostic accuracy of HRCT was reported to be as high as 90% by experienced pulmonologists and radiologists. Herein, we report the case of an elderly woman with Birt-Hogg-Dubé syndrome (BHDS) whose HRCT images displayed lymphangioleiomyomatosis-like features of the pulmonary cysts, rendering it difficult for us to diagnose BHDS. This case illustrates the significance of a thorough anamnesis, physical examination, and skin biopsy of facial papules to establish an accurate diganosis.