Ectodermal dysplasia (ED) is a rare hereditary disease characterized by a congenital dysplasia of several structures of ectodermal origin. Common manifestations include defective hair follicles and eyebrows and deficiency of sweat glands. Intraorally, anodontia, hypodontia or oligodontia are the most common findings. We report the case of a four-year-old girl who was referred to the pediatric dentistry department of hospital la Rabta (Tunisia) and exhibited many of the dental manifestations of ED as well as general abnormalities. Our early oral rehabilitation was launched to improve oral functions and esthetics by including the fabrication of two removable prostheses and acid-etched composite resin restorations. The patient reported a good adaptation of the conventional dental removable prosthesis and parents were satisfied with the treatment.