Relapsing polychondritis (RP) is a rare multisystem disorder characterized by inflammation and destruction of cartilaginous tissue. Atypical initial symptoms and signs may cause difficulties in the diagnosis of RP for pediatricians. In this article, we report a 9-year-old male who presented with stridor and hoarseness indicating upper respiratory tract involvement in the setting of chronic polyarthralgia. Flexible bronchoscopy revealed subglottic stenosis. Increasing awareness of early respiratory manifestations of RP will prevent life-threatening complications.