BACKGROUND: Ectodermal dysplasia affects at least two ectoderm-derived structures such as hair, nails, skin, sweat glands and teeth. The dentition in hypohidrotic ectodermal dysplasia (XHED) is altered with many phenotypes ranging from oligodontia to anodontia. No consensus exists on the ideal age for beginning of prosthetic rehabilitation. CASE REPORT: A 2-year-old male patient with severe oligodontia in the primary dentition was referred for examination, evaluation and treatment. The child exhibited classical XLHED features with sparse hair, absence of eyelashes and eyebrows, severe hypohidrosis associated with heat intolerance and skin dryness. Radiographic examination revealed only intra-osseous maxillary primary canines. He presented an apparent prognathism of partially functional cause, retrusion of the maxillary and lateral deviation of the mandible. TREATMENT: The rehabilitation protocol was similar to an edentulous adult but extra care was taken to avoid discomfort to this very young patient. For example, alginate was used for the impressions for its neutral taste, ease of use, and quick setting speed. An hour was given for the child to adapt to the maxillary prosthesis and then the mandibular one was fitted. Denture adhesive was used to maximize retention. The child succeeded in eating some dry biscuits and drinking some water, demonstrating the exceptional potential for adaptation children usually present with at this very young age. FOLLOW-UP: Three months later, the prognathic tendency had vanished and he could eat hard food. Periodic recall visits were scheduled and at six months and normalization of oral functions was almost complete. Three years later, a new set of prostheses was made very easily as the child knew the protocol and was used to dentures wearing. CONCLUSIONS: Prosthetic rehabilitation must be done at the earliest age possible in order to maintain and correct the oral functions and prevent growth anomalies. It makes the subsequent treatment steps easier. Early rehabilitation and follow-up seems to be one of the keys to a successful treatment that help these children overcome their handicap and integrate them into society.