Dentinogenesis Imperfecta is an autosomal dominant disorder of dentine affecting primary and permanent dentition. Mutation in dentin sialophospho protein on the long arm of chromosome 4 results in this defective dentin. The clinical appearance of teeth is characteristic showing an amber like translucency and severe attrition. It can also occur in association with osteogenesis Imperfecta, a genetic disorder of type 1 collagen. Here we present a case of Dentinogenesis Imperfecta associated with Type 1 Osteogenesis Imperfecta in a 14-year -old girl.