Ectodermal dysplasia exhibits a classic triad of hypohidrosis, hypotrichosis, and hypodontia. Self- mutilation could be due to organic or functional causes. The occurrence of selfmutilation with functional cause represents a diagnostic challenge to practitioners. In most of the instances dentists are the first to recognize patient with ectodermal dysplasia as they report primarily with a complaint of missing teeth. The most common type is hypohidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). A thorough knowledge of this disease with multidisciplinary approach aids in successful outcome of the treatment. This is an unusual case report of Christ-Siemens-Touraine syndrome with selfmutilation.