Ectodermal dysplasia may severely affect the development of jaw growth and facial appearance. This case report describes the treatment of two patients suffering from ectodermal dysplasia, both treated with dental implant-fixed restorations by means of computer-guided surgery. Two patients presented to our clinic with congenital malformation of the jaw as a manifestation of ectodermal dysplasia, showing oligodontia and alveolar ridge deficit. Clinical examination revealed multiple unattached teeth and a need for prosthetic therapy. For both cases, dental implants were placed based on a computer-guided planning. A surgical guide was used to determine the positioning of the dental implants according to the prosthetic planning, which allowed for a satisfactory aesthetic and functional outcome. Computer-guided implant placement allowed predictable treatment of complex cases with satisfactory aesthetic and functional results. Adequate surgical and prosthetic planning is considered critical for treatment success.