This article outlines the dental conditions and treatment findings of individuals ranging in age from 4 months to 30 years of age diagnosed with ankyloblepharon-ectodermal dysplasia-cleft lip/palate (AEC) syndrome. The average number of permanent teeth present is 4.75 with a range of 0–12 teeth. The most frequently present permanent teeth are first molars, canines and maxillary incisors. The dentition of AEC-affected individuals in our study is similar to reports in other ectodermal dysplasia syndromes. The dental treatment findings, including those necessitated by the cleft palate defect seen in the syndrome, are also similar to age appropriate treatment seen in other cases of ectodermal dysplasia syndromes. Only 22% of subjects had prosthetic replacement of missing teeth. History indicated that issues related to treatment of the palatal cleft and lack of recognition of the oral defects as a part of the underlying medical problem were factors in receiving comprehensive tooth replacement care. © 2009 Wiley-Liss, Inc.