The authors describe the ophthalmological findings and clinical course in two patients with EEC syndrome and one patient with anhydrotic ectodermal dysplasia. A retinal complication was seen in a 43-year-old patient which seems to be associated with the EEC syndrome and has not yet been described. A review of the literature indicates the frequency of ocular findings. So far, primary ocular changes in anhydrotic ectodermal dysplasia have never been observed. The older the patient, the more severe the secondary complications affecting the lids and cornea can be; the eye may even be lost.