Regional odontodysplasia (ROD) is a rare developmental anomaly involving both mesodermal and ectodermal components in a group of contiguous teeth. It affects the primary and permanent dentitions in the maxilla and the mandible or both, however, the maxilla is frequently involved. Although the etiology 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 esthetic needs in each case. This article reports the case of a 5-year-old boy presenting a rare anomaly on the right side of the maxillary arch. The treatment performed was rehabilitation with temporary partial acrylic denture and periodic checkups. The extraction was followed by rehabilitation with dental implants. The main aim of this article is to provide valuable information to pediatric dentists about the review and treatment alternatives for ROD.