A case of acrocephalosyndactyly in a 63-year-old woman is reported. The intelligence, sex, and racial distribution, hereditary tendency, and chromosomal analysis of acrocephalosyndactyly is discussed. Ocular complications of this syndrome were noted to be mainly optic atrophy, exophthalmos, and strabismus. Keratoconus, which was found in this patient is uncommon. The patient had a mycotic ulcer of the cornea which was successfully treated with a Gundersen flap.