The patient is a 42-year-old woman. She had taken terguride for proractin-producing pituitary adenoma since the age of 29. She stopped taking it because of pregnancy at the age of 35. After the lactation period, she did not retake terguride, and her blood test has shown that the level of prolactin is above the normal range. She noticed a right abdominal mass and visited our hospital in May 2009. Computed tomography revealed bilateral renal tumors composed of fatty, vascular, and smooth muscle elements in the abdomen, and multiple thin-walled cysts of varying sizes in both lungs. Pathological and immunohistological findings of transbronchial lung biopsy specimens led to a diagnosis of lymphangioleiomyomatosis. There is a report that prolactin may be an important growth factor in the pathogenesis of lymphangioleiomyomatosis. In our case, although she has been suffering from the state of hyperprolactinemia for a long period, progression of the disease is very slow. We considered that our case is contrary to the report that prolactin may promote cell proliferation of lymphangioleiomyomatosis.