Subglottic stenosis (SGS) refers to narrowing of the trachea in the region of the subglottis, directly below the vocal folds and may be congenital or acquired. Acquired SGS has a multitude of causes however iatrogenic following repeated or prolonged intubation is the most common. Autoimmune causes are significantly less common with IgG4-related disease representing a small minority. IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition that has a wide range of presentations and capacity to affect most organs. This disease typically manifests within the pancreas, orbits, salivary glands and retroperitoneum but rarely presents as primary laryngeal disease. We report the case of a 68 year old female with IgG4-related subglottic stenosis. Her primary symptoms were exertional dyspnoea and dysphonia originally diagnosed and managed as vocal cord dysfunction. A subacute deterioration in her symptoms led to her review at a metropolitan laryngology clinic with evidence of glottic-subglottic stenosis on fibreoptic nasendoscopy. Comprehensive laboratory workup including an autoimmune screen were normal however a CT larynx demonstrated a 19mm segment of subglottic stenosis with an intraluminal diameter of 5mm. Surgical management involved radial CO2 laser division, balloon dilatation and steroid injection. Intra-operative biopsies confirmed her unique diagnosis and post-operatively she was initiated on systemic glucocorticoid therapy. A rare pathology, IgG4-related laryngeal disease should be considered in the differentials of patients with subglottic stenosis. Notably, autoimmune markers may be normal and often intraoperative biopsies alone confirm the diagnosis and lead to the initiation of appropriate systemic treatment options.