Regional odontodysplasia (RO) is an unusual, non-hereditary anomaly of the dental hard tissues with characteristic clinical, radiographic and histological findings. Etiology is unknown, and treatment, while varied, should be appropriate for each individual's clinical findings. In the case reported here, a 2-year-old female was diagnosed with RO. Extractions were performed under general anesthesia. Clinical, radiographic, and histological findings are described.