Idiopathic subglottic stenosis (ISS) is a rare inflammatory process of unknown cause, usually limited to the subglottic region and the first 2 tracheal rings. We performed a retrospective analysis of our experience with a series of patients with this condition. The study involved retrospective review of the records of patients with ISS. A series of criteria that included patient clinical history, laboratory tests, flexible nasolaryngoscopic examination, and biopsies were used to establish a diagnosis. Symptoms, treatment, and outcome were examined. A total of 16 patients were identified; 14 were female (87.5%), and 2 were male. The mean follow-up time was 75.5 months. Fourteen patients required surgical treatment for respiratory compromise. Nine of these cases have been controlled effectively with endoscopic laser techniques. Endoscopic management failed in 5 patients, and 2 patients underwent laryngotracheal resection and reconstruction. Mitomycin-C was used in 6 patients as an adjuvant to endoscopic laser surgery. Patients in whom endoscopic treatment failed were noted to have thicker (>1 cm) and more complex stenoses. The diagnosis of ISS is a diagnosis of exclusion. A complete workup of the patient must be performed to rule out other causes of stenosis. Endoscopic laser surgery is a valid initial approach for thinner, noncomplicated lesions. Patients with thicker, complex lesions in whom endoscopic treatment fails are best managed with laryngotracheal resection and reconstruction. However, adjuvant use of mitomycin-C may prove beneficial in the treatment of these patients. Estrogen may play a role in the pathogenesis of ISS by altering the wound healing response.