Regional odontodysplasia is an uncommon dental malformation with characteristic clinical and radiographic findings. Affected teeth appear discolored with irregularly shaped surfaces. Radiographically wide pulp chambers and thin poorly defined hard tissue outlines are typical features. This report describes a 9-year-old girl with recurrent and recalcitrant periapical infections as a result of unilateral mandibular odontodysplasia. With a 6-year follow-up, this is the longest documented case of regional odontodysplasia treated by tooth autotransplantation.