An 83-year-old man presented with a three-week history of dyspnea. The clinical features suggested a diagnosis of relapsing polychondritis (RP); however, the patient died of heart failure. An autopsy revealed active chondritis of the tracheal and bronchial cartilage. Furthermore, giant cell myocarditis (GCM) and myositis were detected. To the best of our knowledge, this represents the first report of RP complicated by GCM and myositis. In patients with RP, GCM and myositis, CD163-positive macrophages and T-cells are most common, and the T-cell subset exhibits CD8 predominance. Common mechanisms of tissue damage caused by cytotoxic T-cells are likely to contribute to RP, GCM and myositis.