This is the first case report in the literature of idiopathic subglottic and tracheal stenosis in a patient diagnosed with multifocal fibrosclerosis (MFS). The patient had unilateral orbital pseudotumour, bilateral pretibal cutaneous manifestations and raised thyroid antibodies, which might indicate subclinical thyroid involvement. Urgent permanent tracheostomy was needed. The patient also underwent tracheal dilatation for her dysphonia. There was no evidence of brain, lung, liver or renal involvement. Although extremely rare, laryngologists should consider the possibility of such a diagnosis in a stridulous patient with multifocal fibrotic manifestations.