Lymphangioleiomyomatosis is a rare multi-system disease that can occur sporadically or be associated with tuberous sclerosis. There is abnormal smooth muscle proliferation with resulting obstruction of lymphatics, venules and small airways. In the lungs, there is development of thin-walled cysts, with surrounding normal lung parenchyma. It can be associated with recurrent pneumothoraces as well as chylous pleural effusions.