EEC syndrome is a multiple congenital anomaly syndrome characterized by ectodermal dysplasia, distal limb anomaly, cleft lip and palate and ocular adnexia anomalies. A case report of a 10 year old female patient with EEC is presented and it is emphasised 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.