Pulmonary lymphangioleiomyomatosis (PLAM) is a rare disease, occurs in 16-68-year-old women, especially in women of childbearing age. High-resolution computed tomography would be useful for diagnosis of PLAM. Immunohistochemistry of smooth muscle actin (SMA) and HMB-45 smooth muscle cells was positive for smooth muscle cells. Progesterone receptor and estrogen receptor in some smooth muscle cells were positive for some smooth muscle cells. HMB-45-positive diagnosis of the disease is more important.