A 49-year-old woman had been treated for 18 months for superior limbic keratoconjunctivitis of the right eye. Pathologic examination of a biopsy specimen of the superior perilimbal conjunctiva disclosed intraepithelial carcinoma of sebaceous gland origin. Because of the extensive involvement of the conjunctival epithelium by tumor, a subtotal orbital exenteration was performed. To our knowledge, this is the first report of sebaceous carcinoma masquerading as superior limbic keratoconjunctivitis.