Abstract: Dentin dysplasia type I (DD-I) is a rare hereditary disturbance in dentin formation. It is characterized by the normal tooth crown but abnormal root development, which is manifested by the presence of short, blunted, and malformed roots; tooth loosening with alveolar abscess occurs at a young age in patients; imaging examination shows occluded pulp cavities. Typical dentin dysplasia type I is autosomal dominant. Patients often loss multiple teeth, and may become edentulous by their 30s. Clinical treatment is challenging. In this paper, a DD-I case is reported, summarizing its clinical manifestations, imaging and histological features, and related treatments in order to provide guidance for the clinical diagnosis and treatment of DD-I.

Key words: Dentin dysplasia type I, Autosomal dominant inheritance, Clinical features, Implant treatment