regional odontodysplasia (ro) is a rare developmental anomaly involving both mesodermal and ectodermal dental components in a group of contiguous teeth. it affects the primary and permanent dentitions in the maxilla and mandible or both jaws. generally it is localized in only one arch. the etiology of this dental anomaly is uncertain. clinically, affected teeth have an abnormal morphology, are soft on probing and typically discolored, yellow or yellowish-brown. radiographically, the affected teeth show a "ghostlike" appearance. this paper reports the case of a 5-year-old girl presenting this rare anomaly on the left side of the maxillary arch, which crossed the midline. the primary maxillary left teeth (except for the canine) and the primary maxillary right central incisor were missing due to previous extractions. the permanent teeth had a "ghostlike" appearance radiographically. the treatment performed was rehabilitation with temporary partial acrylic denture and periodic controls. in the future, the extraction of affected permanent teeth and rehabilitation with dental implants will be evaluated. the presentation of this case adds valuable information to pediatric dentists to review special clinical and radiographic features of ro, which will facilitate the diagnosis and treatment of patients with this condition.