Prosthodontic treatment for patient with hypohidrotic ectodermal dysplasia (HED) is difficult to manage because of the loss of occlusal vertical dimension, abnormal alveolar ridge and anodontia A 20-year-old case with HED was reported. The patient was treated with occlusal reconstruction by a maxillary fixed prosthesis and mandibular telescopic prosthesis. At one-year's follow-up, dentures were well used and occlusal function of the patient returned to normal.