According to the 2017 American Thoracic Society/Japanese Respiratory Society (ATS/JRS) diagnostic criteria for lymphangioleiomyomatosis (LAM), our patient's diagnosis was confirmed through histopathological verification of renal and retroperitoneal angiomyolipomas (AMLs), postoperative development of chylothorax, thoracic CT evidence of bilateral diffuse thin-walled cystic lesions, and exclusion of tuberous sclerosis complex (TSC) via negative TSC1/2 genetic testing coupled with absence of extracutaneous TSC manifestations. Here, TSC1 is linked to Chylothorax.