Pulmonary lymphangiomatosis is a very rare pulmonary lesion with an aggressive potential that occurs mainly in newborns, infants and young children of both sexes. It is characterized by pulmonary abnormalities of lymphatic system, showing an increased number of complex anastomosing lymphatic channels in the pleura, in the subpleural interlobular septa, and along the bronchovascular lymphatic route and uniformly fatal. We report a case of lymphangiomatosis behaving like lymphangioleiomyomatosis in a 26-year-old woman.