Dentin dysplasia is a rare, hereditary disorder affecting the dental hard tissue. It is a congenital, autosomal dominant disease of unknown etiology that affects 1:100,000 populations. It may present as such affecting only the dental hard tissue or as one of the symptoms of underlying diseases such as calcinosis, Ehlers-Danlos syndrome, rhematoid arthitis, Vitaminosis D and Branchioskeletogenital syndrome. This was first described by Ballschmiede as rootless teeth in 1920 and termed as dentin dysplasia by Rushton in the year 1939. It is classified into Type I, II and III, in which Type III affects only the secondary dentition. This article reports a rare case of Type I dentin dysplasia in a 26-year-old male patient, and focus on clinical, radiological, ground section and histopathological aspects. It emphasizes the significance of early diagnosis and intervention for the psychological well-being of the individual.