A rare case of unilateral cryptophthalmos have unique findings is reported. The lateral part of the left eye was replaced by downward extension of the frontal hairline. The left upper eyelid, except in the lateral 4mm fully developed portion, was replaced by a fold adherent to the underlying disorganized globe (6.9 mm). The lower lid, however, was fully developed. Ocular and systemic features of cryptophthalmos have been reviewed. Predilection for the left eye in unilateral cases has been hinted for the first time.