We retrospectively reviewed our experience with catamenial pneumothorax (CP) in terms of treatment and follow-up. From 1993 to 2008, ten women presented at our department with CP. CP was right-sided in all patients: seven presented diaphragmatic defects including one endometriosis, five had apical bulla or blebs that in three patients were the only pathological findings. Surgical approach was thoracoscopic with a muscle-sparing thoracotomy when diaphragmatic defects where present. All patients underwent apical resection and apical pleurectomy associated in seven cases with diaphragmatic plication and chemical pleurodesis. After surgery nine patients underwent hormonal treatment: three were put on estrogen-progesterone complex treatment and six received gonadotropin-releasing hormone agonist (GnRH agonist). Recurrence rate was 40% and it was significantly correlated with estrogen-progesterone treatment (P<0.005). The mean follow-up was 52+/-32 months (range 14-168). At the present time, no recurrence has occurred in all women. Occurrence of CP is often underestimated. At the time of surgery the diaphragm should be carefully inspected for defects and/or endometriosis. Standard pleurodesis may not suffice and we suggest apical resection and apical pleurectomy associated with a diaphragmatic procedure when indicated. Hormonal treatment with GnRH agonist seems to improve the outcome.