Relapsing Polychondritis (RP) is a systemic disorder characterized by an inflammatory process involving predominantly cartilaginous structures and the cardiovascular system. Lymphadenopathy is a very uncommon finding of RP. We report on a patient affected by RP presenting with lymphadenopathy of Castleman-like type quickly responsive to corticosteroids. The bronchial involvement and the evolution of the inflammatory process in a 3-year follow-up has been documented by computed tomography of the chest.