Purpose: To report a case of unilateral corneal hydrops associated with vernal conjunctivitis as a presenting sign of keratoconus.

Method: Case report. A 10-year-old girl with corneal hydrops of the left eye.

Result: Visual acuity in the affected eye was hand motions and slit-lamp examination revealed a left keratoconic, ectasis, edematous cornea with mild vernal conjunctivitis.

Conclusion: Hereditary and environmental factors may contribute to the etiology of keratoconus and corneal hydrops.

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