A case of dentinal dysplasia type I is presented. This rare hereditary disturbance of dentine is characterized by short-rooted teeth with sharp conical apical constrictions, aberrant growth of dentine in the pulp chamber leading to reduced pulp space in permanent teeth and total pulpal obliteration in the primary dentition. Clinical, radiographic and histopathological material from a 7-year-old boy, showing the typical features of this disorder in which teeth are prematurely lost through periapical abscesses, cysts or spontaneous exfoliation, is described. A review of the theories of pathogenesis of this condition is included. Management of patients with dentinal dysplasia is difficult and a discussion of the shortcomings of various treatment strategies, including conventional endodontic therapy, periapical curettage and retrograde root filling, and a preventive regimen, are discussed. In this case, despite diagnosis being made at an early age and the provision of regular dental care, the patient is now losing teeth because of spontaneous abscess formation.
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