Ectoderma dysplasia is a syndrome characterized by malformation in the tissues derived from the embryonic leaflet ectoderm, causing changes in the epidermis and its accessory structures, such as hair, hair, nails, teeth and glands. The dental surgeon, in most cases, is the first to suspect this syndrome due to dental absences, which can vary from simple agenesis or even anodontics. Therefore, the alveolar bone does not develop very well and the ridge has an unfavorable morphology for a good rehabilitation. The present work aims, through a clinical case, to describe the difficulty of prosthetic rehabilitation in a patient with Ectoderma Dysplasia in preschool age (3 years), due to the absence of teeth. The treatment defined for this patient was the protocol proposed in the most current bibliographic references, which consists of rehabilitation as early as possible, through the manufacture of upper and lower removable prostheses, in order to enable better nutrition, phonetics, facial expressions and social interaction. After the finalized treatment, we conclude that the early prosthetic rehabilitation, although recommended in the literature, must first be evaluated individually, as it depends on the minimal cooperation of the patient and total family commitment for the success of the treatment.