BACKGROUND Granulomatosis with polyangiitis (GPA) is associated with development of subglottic stenosis in about one-fourth of all patients. Although endoscopic management is the primary treatment method for tracheobronchial stenosis, some patients have refractory disease, and tracheostomy is required. It is unclear if laryngotracheal resection and reconstruction (LTRR) can be safely performed in patients with GPA. METHODS A retrospective review was performed of 11 patients with GPA undergoing LTRR. RESULTS Eleven female patients with GPA and a median age of 47 years underwent LTRR. Six patients were diagnosed with GPA after LTRR and had not received any induction immunosuppression regimen. Five patients had received induction immunosuppression regimen and were in clinical remission before LTRR. LTRR was performed with a protective tracheostomy in 3 patients, which was eventually removed in all. There were no major complications and no postoperative deaths. One patient (9%) failed surgical management and had replacement of a permanent tracheostomy 4 months after LTRR. Six patients (55%) required additional tracheal dilations after LTRR. Ten patients (91%) had durable control of symptoms and freedom from tracheostomy with a median follow-up of 9.7 years. Two patients (18%) experienced subsequent lower airway stenoses. CONCLUSIONS Surgical treatment of subglottic stenosis in highly selected patients with GPA is effective and associated with minimal morbidity. Although long-term outcomes are encouraging, additional procedures may be necessary, and patients are at risk of experiencing lower airway disease.