Abstract
Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease. Chylous effusion is one of the most common clinical manifestations of LAM. Herein we present a 39-year-old female who presented progressive dyspnea on exertion and chylothorax. The chest computed tomography showed multiple thin-walled cysts in both lungs which suggesting LAM. The pleural effusion cytology plus with further immunocytochemistry confirmed the computed tomography diagnosis. Therefore, the LAM can be diagnosed by cytologic examination combined with conventional chest computed tomography and clinical manifestations, which can help some patients to avoid an invasive biopsy.