Ectodactly Ectodermal Dysplasia (EEC) syndrome is a rare syndrome with multiple congenital anomaly, but with normal intelligence characterized by ectodermal dysplasia, clefting of hands , feet, lip and palate. It is an autosomal dominant syndrome. It is emphasized that management of these cases requires a multidisciplinary approach. Early diagnosis will allow parents to get accurate counseling and in particular obtain reassurance regarding the low risk of mental handicap. Here we report a case delivered at maternity and Children’s Hospital, Najran, Saudi Arabia. Examination of that baby revealed ectrodactly (splitting of hands and feet), ectodermal dysplasia, cleft lip and palate, sparse scalp hairs and eye brows and absence of eye lashes. These abnormalities constitute the EEC syndrome.
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