Amelogenesis imperfecta (AI) is a term for clinically and genetically heterogeneous group of conditions affecting the dental enamel, occasionally in conjunction with other dental, oral and extraoral tissues. It is usually inherited either as an X-linked, autosomal dominant or autosomal recessive trait. The enamel may be hypoplastic, hypomineralised or both and affected teeth may be discolored, sensitive or prone to disintegration.Diagnosis is based on the family history, pedigree plotting, meticulous clinical and radiological observation.Dental radiographs of AI teeth provide important information to the clinician with respect to the degree of enamel mineralization to design an appropriate treatment plan. The treatment of patients with AI should start with early diagnosis and intervention to prevent latter restorative problems. Herein, we present a case report of hypomaturation amelogenesis imperfecta with oligodontia and taurodontism who were provided with functional and esthetic rehabilitation.

Keywords: Amelogenesis imperfecta; Enamel; Genetic; Hypomaturation.