Hereditary benign intraepithelial dyskeratosis (HBID) is a rare disorder characterized by autosomal dominant inheritance, onset in childhood, bilateral limbal conjunctival plaques, chronic relapsing course of ocular irritation and photophobia, and oral lesions resistant to medical and surgical therapy. The lesions are characterized histologically by dyskeratosis, acanthosis, parakeratosis, and a variable amount of subepithelial inflammation. The patients in all previously reported cases have been descendants of Halowar Indians from North Carolina. This report illustrates the typical clinical and histologic findings of HBID in two siblings who, along with their parents and grandparents, were born in Texas.