The aim of this study was to report a case of isolated corneal squamous cell carcinoma in a patient with unilateral dry eye symptoms. The case was a 48 years old man with history of thermal corneal injury on his left eye two years ago with decreased visual acuity and dry eye symptoms a short time after, which was treated with the diagnosis of dry eye and had no improvement in symptoms. Examination revealed hypertrophy of near total corneal epithelium, without limbal and conjunctival involvement. The surface of the lesion was irregular with punctate epithelial erosions. The patient was underwent excisional biopsy which was revealed severe corneal dysplasia (carcinoma in situ). After excision, the patient was treated with Fluorouracil and there was no sign of recurrence till the time of last follow-up examination. Dry eye symptoms were disappeared completely. We conclude that, in the case of unilateral chronic dry eye which was resistant to the treatment with signs of corneal epithelial involvement, isolated corneal neoplasia should be considered even without limbal or conjunctival involvement.