Embodiments disclosed herein provide compositions and methods for treating lymphangioleiomyomatosis (LAM) comprising inhibiting COX overexpression and prostaglandin over production by administering at least one COX inhibitor and/or prostaglandin biosynthetic pathway inhibitors. Lymphangioleiomyomatosis (LAM) is a rare lung disease. Some LAM occurances are associated with mutations in the tuberous sclerosis complex (TSC) locus. LAM occurs almost exclusively in women, usually of childbearing age.