PURPOSE: This documents the evaluation and management of a patient with an unusual destructive external ocular disorder in the context of progressively disfiguring dermatitis. METHODS: Observational case report including ophthalmologic examination with conjunctival and corneal scraping, radiologic studies of the sinuses and chest, skin, conjunctival, and scleral biopsies, and serologic studies. RESULTS: Biopsies, cultures, and serologic testing led to the conclusion that the patient had pyoderma gangrenosum. Therapy with dapsone resulted in preservation of vision and resolution of the oculocutaneous inflammation. CONCLUSIONS: Pyoderma gangrenosum is a rare autoimmune disorder that can affect the eye. Biopsy of affected tissue is the key to diagnosis. Immunomodulating therapy can be a useful adjunct to the traditional steroid therapy.