Regional odontodysplasia (ROD) is a rare, localized developmental anomaly of the dental tissues with distinctive clinical, radiographic, and histologic findings. It affects the primary and permanent dentitions in the maxilla and the mandible or both, however, the maxilla is frequently involved. Although the aetiology of the ROD is uncertain, it has been suggested that numerous other factors play a role. The treatment plan should be based on the degree of involvement as well as the functional and aesthetic needs in each case. Here we describe a case of regional odontodysplasia in an 8 year old boy with an overview of aetiology and treatment modalities. The main aim of this article is to provide valuable information to dental community about the review and treatment alternatives for ROD.