Lymphangioleiomyomatosis (LAM) is a rare idiopathic disease predominately affecting women. The disease is characterized by peribronchial, perivascular, and perilymphatic proliferation of smooth muscle like cells resulting in vascular and airway obstruction and cyst formation. The natural history of pulmonary LAM is unknown, and it displays remarkable diversity in its clinical course. More than 400 citations are currently listed in the literature for LAM. This article reviews and updates the rapidly expanding knowledge about LAM.