The lack of tooth formation or dental dysmorphic morphology is one of the phenotypes of various forms of ectodermal dysplasias (ED). Teeth that do form are often altered in shape and contour and occasionally manifest with delayed eruption. The oral health care team needs to work with affected individuals starting around the first year of life with age-appropriate and staged oral health interventions through adulthood. For most patients with ED, definitive rehabilitation (with or without implant treatment) should be delayed until skeletal maturity. Having said this, starting around age 2 to 3, pediatric removable prosthetics are appropriate. As a part of the definitive reconstruction as a young adult, the team needs to plan for maintenance issues and complications and use a defined set of diagnostic risk factors in the assessment of the affected individual. Care often involves pediatric dentists, orthodontists, surgeons and prosthodontists to guide each stage. Through careful assessment, planning, team communication, and partnership with the affected individual and her/his family, a successful long-term solution can be developed that provides the best patient-oriented outcomes.