Caveolae are flask-shaped plasma membrane invaginations formed by constitutive caveolin proteins and regulatory cavin proteins. Caveolae harbor a range of signaling components such as receptors, ion channels and regulatory molecules. There is now increasing evidence that caveolins and cavins play an important role in a variety of diseases. However, the mechanisms by which these caveolar proteins affect lung health and disease are still under investigation, with emerging data suggesting complex roles in disease pathophysiology. This review summarizes the current state of understanding of how caveolar proteins contribute to lung structure and function and how their altered expression and/or function can influence lung diseases.