Amelogenesis imperfecta (AI) represents a group of genetic disorders characterized with generalised enamel deffects, which includes all, or almost all, teeth of both dentitious. AI is caused by mutations of five genes; amelogenin (AMELX), enamelin (ENAM), matrix metalloproteinaise-20 (MMP20), kallikrein-4 (KLK4) and FAM83H. The anomaly can be inherited by autosomal dominant, autosomal recessive or X-related (sex) type inheritance. Classification by Witkop and Sauk describes four forms of anomaly: hypoplastic, hypomaturation, hypocalcified and hypomaturation – hypoplasia form combined with taurodontism. AI diagnosis is based on clinical and genetic diagnostic procedure. Detecting the exact diagnosis allows elimination of other disorders with similar clinical picture. Besides the poor dental esthetics, clinical problems are manifested in chewing and speaking dysfunction, dental hypersensibility and in the loss of the vertical dimension of occlusion. AI therapy requires a multidisciplinary approach in periods from childhood to adult age. The selection of restorative procedure depends on the age of a patient, phenotype of anomaly, motivation, periodontal condition, endodontic teeth status and financial status of the patient. Therapeutic options include minimally invasive procedures such as teeth whitening and microabrasion, then restorative procedures with resin composite or glassionomer materials and prosthetic solutions such as ceramic veneers, inlays, onlays, overlays or crowns. In the primary dentition, the dental treatment is reduced to ensure favorable conditions for the eruption of the permanent teeth. During the mixed dentition, the treatment goals are to preserve tooth structures, maintain tooth vitality, decrease tooth sensitivity, establish correct interproximal and oclusal function and improve esthetics. In the permanent dentition, the final treatment proceedings are to diminish tooth sensitivity and to restore vertical dimension of occlusion and function, as well esthetics.