Purpose: The aim was to report on oral rehabilitation of a boy with X-linked hypohidrotic ectodermal dysplasia (XLHED) and anodontia of the mandible between ages 3 and 33 years where treatment involved dental implants and oral care management by a multidisciplinary team of specialists. Materials and Methods: This case history report describes the clinical management of a boy born in 1979 with XLHED and anodontia of the mandible. Two implants were successfully placed in the anterior region of the mandible in 1985. Genetic analysis later verified the diagnosis by confirming a mutation in the EDA gene. The case description was based on review of the patient records and assessment of orofacial function. Results: The patient had satisfactory orofacial appearance and function throughout his youth. He is still caries-free at age 33 and has experienced only minor oral complications. Dental management began at age 3, when he received a maxillary removable dental prosthesis. At age 7, he received a mandibular implant-supported overdenture. After two more implants in the mandible and orthodontic treatment in the maxilla, his oral rehabilitation was completed at age 22 with maxillary tooth-supported and mandibular implant-supported fixed dental prostheses. Regular follow-ups provided supervision of oral hygiene, caries prevention, and prosthetic maintenance. Conclusion: This long-term follow-up of a child with XLHED and anodontia in the mandible supports the use of dental implants, with consideration given to the dense bone quality associated with the diagnosis, to establish good orofacial function and appearance from childhood onward.