Abstract
Dentinogenesis imperfecta (DI) is a hereditary condition which affects the development of dentine in both the primary and permanent dentitions. Three types of DI have been described in the literature. The presentation of DI is variable, depending on the type and severity of the disease. Early intervention in the treatment of a patient with DI is extremely important both for psycho-social and for functional reasons. This paper attempts to describe the clinical presentation and management of DI, and explores its association with certain medical conditions.