Ectodermal dysplasia, which affects ectodemal derivatives, currently is an X-linked recessive inheritary hypohydrotic ectodermal dysplasia. Patients with ectodermal dysplasia usually characterized by congenital teeth loss, abnormal teeth and inherited through female carriers. In this case report, we present a 5 years old boy of China ethnicity with mandibular anodontia, a rare dental dysplasia in ectodermal dysplasia syndrome. To compromise esthetics and function, a multidisciplinary approach was performed by oral physician, oral pathologist, orthodontist and prosthodontist.