Background <p>Ectodermal dysplasia (ED) presents significant dental and craniofacial challenges, particularly in prosthetic rehabilitation due to anodontia or oligodontia, underdeveloped alveolar ridges, and altered vertical dimensions. Although diverse treatment modalities have been described, no consensus exists on the most effective rehabilitation protocol.</p> Aim <p>To systematically review the literature on prosthetic rehabilitation in patients with ED, encompassing removable dental prostheses, fixed dental prostheses, and implant-supported prostheses, and to evaluate outcomes in terms of survival, function, aesthetics, patient satisfaction, and complications.</p> Material and methods <p>A comprehensive manual and electronic search of PubMed (MEDLINE), Web of Science, Scopus, and Google Scholar (grey literature) was conducted. Clinical studies, case series, and case reports involving prosthetic rehabilitation in ED patients were included. Data extraction was performed independently, and risk of bias was assessed using ROBINS-I and the JBI Critical Appraisal Checklist.</p> Results <p>Twenty-nine studies were included, comprising case reports, case series, and prospective and retrospective cohort studies. Treatment approaches varied widely, including removable dental prostheses, fixed dental prostheses, and implant-supported prostheses. Most studies emphasised early intervention, multidisciplinary planning, and periodic revision of prostheses during growth. Implant-supported prostheses showed favourable long-term outcomes in adolescent and adult patients. Risk of bias ranged from low to moderate across studies.</p> Conclusion <p>Prosthetic rehabilitation in ED requires an individualised, multidisciplinary approach. While implant-supported prostheses demonstrate promising outcomes, early removable solutions remain essential in paediatric cases. Further prospective studies with standardised protocols are needed to guide evidence-based treatment strategies.</p>