Ectodermal dysplasia is a rare congenital hereditary entity. The most commonly observed forms of ectodermal dysplasia are the hidrotic and hypohidrotic types; discrimination is based on the absence or presence of sweat glands. In this report, a child with hypohidrotic ectodermal dysplasia having true anodontia of the primary dentition is presented. Physical, mental, and intraoral examinations were performed. Photographs and occlusal and panoramic radiographs were taken. A medical consultation and biopsies were requested. Mentally, the patient was normal. His hair and eyebrows were light colored and sparse. He had frontal bossing, a saddle nose, reduced vertical facial dimension, and prominent supraorbital ridges and chin. Radiographs revealed absence of all primary and permanent teeth except the bilaterally unerupted maxillary permanent canines. An armpit biopsy specimen revealed the absence of eccrine and apocrine glands. A removable prosthesis was made to satisfy the patient's esthetic and functional needs. The absence of primary teeth (true anodontia) is a rare phenomenon. It is claimed that primary teeth must be present for the development of their permanent successors. In the present case, however, the permanent canines existed despite the absence of their predecessors. Dental clinicians can be the first to diagnose ectodermal dysplasia. The dental team should be aware of its signs and symptoms in order to provide the correct therapies for the functional and psychologic needs of these patients. Keywords: anodontia, hypohidrotic ectodermal dysplasia, primary dentition