A search of the literature over the past 150 years reveals 19 proband cases of lobster claw deformity of the extremities and clefting of the primary and secondary palate. Some authors have reported that a few of these cases also have an atypical form of ectodermal dysplasia in which the skin is fair and the hair fine and light colored, and a tooth enamel dysplasia but normal salivary, sweat, and sebaceous gland function. Atresia of the lacrimal puncta may also be a part of this syndrome which has been designated the ectrodactyly‐ectodermal dysplasia‐clefting (EEC) syndrome. This report describes two unrelated females who appear to have the EEC syndrome, thereby bringing the total probable cases to five, two of which occurred in affected families (Walker & Clodius 1963, Cockayne 1936) and three of which were sporadic (Rudiger et al. 1970, this report). Dominant inheritance with incomplete penetrance is suggested.

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