The regional odontodysplasia, also referred as odontogenic dysplasia, is a rare condition which has been described as an alteration of the dental development affecting the mineralized structures of both the permanent and deciduous teeth, and it has been associated with retardation in the eruption of the affected teeth, gum edema, hydrocephalus and Epidermal Nevus Syndrome. The etiology is still unknown, however, some hypotheses have been suggested about their origin. About its incidence, this disease is more frequent in the anterior segment of the maxilla and usually unilateral. Epidemiologically this disease shows no predilection for any racial group, being more frequent in the first and second decade of life and predominantly in females. Clinically the Odontodysplasia Regional is characterized as an hypoplasia of enamel and dentin in teeth badly formed with size alteration and form coloration varying of the yellow to chestnut; the presence of smooth and extremely friable dentine, tooth mobility and defective mineralization increases the possibilities of the pulp exposure and a tendency to develop abscesses. Radio-graphically has the appearance of ghosts tooth with a bizarre morphology; reduction of the normal radiopacity decrease of the thickness of the dentine and enamel structures. They generally present with open apexes; shortened or absent roots; diffuse calcification in the inside of the pulp. Histopathologically it presents with accented reduction of the amount of dentin, presence of great interglobular dentine areas and irregular standard of the dentine canals. Enamel is usually thin, hypoplasic and hypocalcified. The pulp presents atrophic areas, fibrose and hyalinization and the cement does not have marked structural alterations. The treatment of the Regional odontodysplasia has been the reason of many quarrels. This present essay aims to review the literature and report a clinical case.