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Regional odontodysplasia (RO), also known as ghost teeth, is an unusual nonhereditary developmental anomaly of tooth formation that characteristically affects enamel and dentin formation of the primary and/or permanent dentition. In the present paper, we report a case of RO affecting a 7-year-old boy, with 9 years of follow-up. During this time, progressive development of dental tissue was observed, including complete root formation. However, delayed dental eruption was evident. In view of these findings, we discuss the clinical presentation, pathogenesis, differential diagnosis, and treatment of RO.