Agenesis of the permanent dentition is rare. This report describes a 20-yearold woman with 19 deciduous teeth, a single permanent mandibular premolar, and other physical traits associated with ectodermal dysplasia. The patient demonstrated esthetic parameters associated with maxillomandibular alveolar insufficiency, and her chief complaints were directed toward esthetics and the potential impact of restorative choices on function. Three typical options for restoration include overdentures, removable partial dentures, or implantsupported prostheses replacing her natural dentition. This report illustrates a fully integrated digital approach to treatment planning, the fabrication of a computer-aided design/computer-assisted manufacture surgical guide and provisional restoration, guided implant placement, and definitive restoration using monolithic zirconia implant-supported fixed dental prostheses. The lifelong management of this rehabilitation is an acknowledged challenge.