Thoracic endometriosis syndrome includes four well-recognized clinical entities, namely catamenial pneumothorax, catamenial hemothorax, catamenial hemoptysis and lung nodules, as well as some exceptional presentations. The etiological mechanisms of this syndrome are not well understood, and different theories have been proposed. Controversies exist about optimal management, as experience has been drawn from case reports and small clinical series. Surgery, hormonal treatments and combined approaches have all been proposed, with variable results in terms of short and long term outcome.