Ectodermal dysplasia is characterized by a partial or complete lack of primary and permanent teeth, other ectodermal structures that may be affected include the skin, hair, and sweat glands. The patient with the so-called anhidrotic type of ectodermal dysplasia exhibits dry skin, lack of sweat glands, sparse eyebrows, body hair, saddle nose, and everted lips. Genetic basis of anhidrotic ectodermal dysplasia is recessive and sex-linked, being manifested chiefly in males, but this is debatable. A 6-year-old boy, with typical signs of anhidrotic ectodermal dysplasia, was presented. Prosthetic restoratoins are of great value to these patients, both from the standpoint of function and for psychologic reasons. The need for complete denture is critical during preschool periods and continues into adulthood. The following case report is an approach to the management of a patient with anhidrotic ectodermal dysplasia.