Hay-Wells syndrome or AEC (Ankyloblepharon, Ectodermal dysplasia and Cleft lip and
palate syndrome) is a rare ectodermal disorder. The treatment is aimed to prevent
clinical complications. We describe the case of a four-month old male patient with
erosions on the scalp, trunk and arms, trachyonychia, deformity of the ears,
micropenis, cleft palate, decreased eyebrow and eyelash hairs, in addition to
antecedents of surgical correction of ankyloblepharon. The importance of the correct
diagnosis is emphasized, besides the investigation of the associated diseases,
treatment of complications and genetic counseling of the parents.