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Relapsing polychondritis (RPC) is a rare auto-immune mediated inflammatory disease that can involve cartilage. RPC is known to cause chondritis, perichondritis, arthritis, conjunctivitis, and oculo-nasal deformities. RPC can show severe clinical manifestation especially when it involves laryngeal framework or tracheal cartilage. In such cases, tracheotomy is mandatory to secure airway. It is important to be well-informed of clinical manifestation of the disease for head and neck surgeons to deal with emergency situations, such as acute upper airway obstruction. We introduce a case of RPC, encountering critical situation during tracheotomy