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Aim: Dentinogenesis imperfecta (DI) is an autosomal dominant genetic disease that affects both deciduous and permanent teeth, with an incidence of 1 out of 6,000 to 1 out of 8,000. Teeth affected with DI type II present bulbous crowns, short and constricted roots, marked cervical constriction, translucent enamel and amber dentin. Also, they present a partial or total obliteration of pulp space, due to continuous dentin production. SEM analysis has shown an undulated dentin-enamel junction (DEJ) with irregularities and locally wide spaces between the two structures instead of a strict junction and a regular linear surface. Treatment options for patients affected by DI-II are intended to protect and restore function and aesthetics of both posterior and anterior teeth. In literature are presented many different therapies, but mainly centered on cemented prosthetic restorations instead of adhesive restorative procedures. We present in this paper a DI-II case successfully treated in 2005 with extensive adhesive rehabilitation., Conclusion: The 13 years follow-up proves the reliability of adhesion to dentine and enamel for indirect adhesive restorations even on this kind of anomalous substrates.