Up to 92% of patients with granulomatosis with polyangiitis (GPA) have concurrent ENT manifestations of the disease. Whilst we are familiar with sinonasal and middle ear presentations of GPA, subglottic stenosis (SGS) is another important and potentially life threatening manifestation. SGS is present in up to 23% of patients with GPA and occurs more commonly in younger patients. It can occur in isolation and be the presenting feature of GPA or can occur late. Stenosis is often limited to the subglottis / proximal trachea but it may also extend distally into the proximal bronchi or affect more proximally at the larynx or glottis. The course of SGS with GPA appears to run independently from the systemic disease. This study from a single centre identified 35 patients with both GPA and SGS. Diagnosis of GPA was made using either tissue biopsy, ANCA serology or a combination of both. In 31% SGS was the presenting manifestation of GPA. The remaining patients developed SGS at a median of 2.5 years from diagnosis. Thirty-four percent had multi-level airway involvement with 23% developing vocal cord paralysis, usually from fixation at the cricoarytenoid joint. All but one patient had concurrent head and neck manifestations of disease in addition to SGS. This paper highlights that GPA should be a differential diagnosis for any patient with SGS, without a clear history of airway trauma. The authors advise on the basis of their single centre study that patients with GPA and SGS should undergo bronchoscopy at the time of any airway intervention or trachea-bronchoscopy performed regularly in any tracheostomy dependent patient.