Relapsing polychondritis (RP) is a rare immune-mediated disease affecting cartilaginous structures. Respiratory tract manifestations are frequent and constitute a major cause of morbidity and mortality. The present review of the literature was designed to assess the efficacy of tumor necrosis factor alpha (TNF-a) inhibitors in respiratory tract involvement of RP. A MEDLINE literature search was performed from January 2000 to December 2016 to identify all studies and case reports of anti-TNF-a therapy in RP. Articles published in English or French concerning patients with respiratory tract involvement were eligible. Two authors (JB, FL) independently reviewed and extracted data concerning each patient and 2 personal cases were added. Treatment efficacy was assessed according to systemic and/or respiratory criteria. A total of 28 patients (mean age: 41.6 years; 16 females/12 males) were included in the final analysis. Anti-TNF-a therapy was associated with improved health status and respiratory symptoms in 67.8% and 60.1% of cases, respectively. These results suggest that TNF-a inhibitors could be considered for the treatment of respiratory tract involvement of RP. Abbreviations: CT = computed tomography, FDG/PET = fluorodeoxyglucose positron emission tomography, RP = relapsing polychondritis, TNF-a = tumor necrosis factor alpha.