An 11-year-old girl developed peripheral corneal thinning and superficial pannus formation with no other ocular or systemic signs. During the subsequent two years she developed inflammation of both ears, progressive collapse of the nasal cartilage, and polyarthritis. A clinical diagnosis of relapsing polychondritis was made. A spontaneous perforation of her right eye secondary to progression of peripheral ulcerative keratitis was successfully repaired. Treatment with dapsone, a nonsteroidal anti-inflammatory agent resulted in remission of her ocular and systemic disease.