Regional odontodysplasia is a developmental anomaly that affects the primary and permanent dentitions. This disorder is generally localized in only one arch and its etiology is still unknown. Clinically, the affected teeth have an abnormal morphology and are typically discolored. Radiographically, these teeth show a ghost-like appearance. This paper reported the results of radiographic, histologic and laboratory findings about the case of a 5-year-old girl presenting this rare anomaly. Her familial history was negative for any genetic anomaly, regional odontodysplasia or other dental anomalies. The patient’s general health was good and no congenital or acquired disease was reported. She was kept under follow-up care until she reached the age of 10 years. Panoramic radiograph showed the involvement of permanent teeth on the right maxillary quadrant. The affected edentulous quadrant was rehabilitated with temporary acrylic maxillary partial denture. The presentation of this case would hopefully have valuable information for pediatric dentists to review the clinical and radiographic features of regional odontodysplasia, yet expediting the diagnosis and treatment of patients with this condition. Keywords ● Regional Odontodysplasia  ● Ghost Teeth  ● Dental Dysplasia