An 8-year-old boy with symptoms of relapsing polychondritis for five years developed retroperitoneal fibrosis and ureteral obstruction. In addition to involvement of the cartilage of his joints, ear, and nose, there was significant arterial involvement. The patient had a very good response to adrenal corticosteroid therapy. After one year of follow-up, he has had a definite increase in joint mobility and has normal renal function.