Abstract We report the case of a 56-year-old Japanese man with Vogt-Koyanagi-Harada disease in whom pain and diffuse swelling of the left auricle and bilateral episcleritis developed 3 years after diagnosis. Biopsy of the left ear showed acute chondritis, leading to another diagnosis of relapsing polychondritis. Additionally, he was found to carry human leukocyte antigen DR4, which has been reported to be associated with these inflammatory conditions. To our knowledge, our patient is the first reported case of the occurrence of relapsing polychondritis and Vogt-Koyanagi-Harada disease.