The case of a 10-year-old girl is reported, in whom the diagnosis of Crouzon's disease was established, with obvious radiological cranial changes, minimal facial abnormalities and at the ocular examination a moderate bilateral exophthalmos, divergent strabismus at the right eye and bilateral optic atrophy with a useful vision only at the left eye, without other associated manifestations and with maintenance of an average intellectual and mental level. The differential diagnosis of the disease, the ocular symptomatology and the treatment are discussed.