A 50-year-old woman with a chief complaint of abdominal pain presented at another hospital. She showed numerous thin-walled lung cysts and abdominal lymphadenopathy on computed tomography. A biopsy of abdominal lymph nodes was performed at the other hospital, and she was diagnosed as angiomyolipoma. Lastly she was diagnosed as lymphangioleiomyomatosis. After a biopsy of abdominal lymph nodes, she had considerable chylous pleural effusions and ascites accumulation. She was treated with a fat-restricted diet and drainage; however, the chylous pleural effusions and ascites were refractory. After she was transferred to our hospital, we began sirolimus therapy with 1 mg/day. Nine days after beginning sirolimus, the chylous pleural effusions and ascites were reduced immediately. Mild stomatitis, rash, and hyperlipidemia were complications resulting from the sirolimus treatment, but we could control these complications. She has currently received sirolimus treatment for approximately 9 months, and the chylous pleural effusions and ascites have not recurred. In this case, sirolimus treatment resulted in the cessation of frequent drainage of uncontrolled pleural effusion and ascites.