A 54-year-old woman was admitted because of an abnormality in the chest X-ray. At the age of 17, she had been diagnosed as Turner syndrome and received female hormone replacement therapy. Although she had no symptom, the chest CT showed multiple small nodular shadows in bilateral lung fields. Laboratory data showed mild elevation of ProGRP. Video-assisted thoracoscopic lung biopsy was performed. Histologically these nodules contained cell foci with pathognomonic whorl formation. Immunohistochemically, the cells were positive for EMA, vimentin, and progesterone receptor, whereas negative for cytokeratin marker. Ultrastructurally, complex interdigitating cell processes were connected by desmosomes. The diagnosis was "diffuse pulmonary meningotheliomatosis," which contained diffuse multiple "minute pulmonary meningothelial-like nodules." During 4 years of observation, interval changes in the size and distribution of the nodules have not been detected. It is necessary to consider this disease when we encounter multiple nodular shadows.