PURPOSE: The aims of this article are to critique the available literature on dental implants in patients with ectodermal dysplasia (ED) syndrome and tooth agenesis, review the outcomes of implant therapy in these patients, and provide recommendations on the timing of implant placement for these patients. MATERIALS AND METHODS: Searches were performed using Medline, Embase, All EBM Reviews, and Pre-Medline for articles relating to implant patients suffering from ED. Articles unrelated to the topic of dental implants in patients with ED and tooth agenesis, without abstracts, or in languages other than English were excluded. Selected articles were graded according to levels of evidence based upon guidelines set forth by the Agency for Health Care Policy and Research. Articles found to have a level of evidence of IV were excluded from this study. RESULTS: The literature on dental implants in patients with ED and tooth agenesis was found to be scarce. No randomized controlled or case-controlled studies were found. Only 12 articles were found to satisfy all inclusion criteria. CONCLUSION: Implant survival rates vary between 88.5% and 97.6% in patients with ED and between 90% and 100% in patients with tooth agenesis. Implants placed in adolescent ED patients do not have a significant effect on craniofacial growth, while implants placed in ED patients younger than 18 years have a higher risk of failure.