Ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC) is a rare genetic disorder. EEC was first described in 1970 and occurs in 7 of 10,000 people. This paper discusses the effects of EEC and the dental complications typically associated with this syndrome. It is important for dental practitioners to be aware of EEC due to the number of dental conditions associated with it. A case report is presented to discuss the dental management, especially issues related to oral hygiene, of a patient who had EEC.