We describe our experience in the diagnosis and treatment of idiopathic subglottic stenosis (ISS), a rare pathological entity involving the subglottic larynx and the first tracheal rings and affecting virtually only females. Its diagnosis can be made only after the exclusion of all other known causes of subglottic stenosis. In a 17-year period, between January 1986 and June 2002, 30 patients were admitted and treated for ISS at the Department of Otolaryngology and/or the Center for Respiratory Endoscopy and Laser Therapy, the University of Brescia, Italy. Clinical, endoscopic and surgical records were retrospectively analyzed with particular emphasis on treatment (endoscopic versus open-neck procedures) and follow-up. Based on our experience, we can define endoscopic treatment by carbon dioxide or Nd:YAG laser-assisted dilatation and scar tissue resection with or without airway stenting as the treatment of choice for the initial management of ISS. After repeated endoscopic failures, open-neck surgery by laryngoplasty or laryngotracheal resection and anastomosis is strongly recommended, particularly for complex lesions longer than 1 cm.