Dens evaginatus (DE) and dens invaginatus (DI) are rare developmental dental anomalies affecting both the primary and permanent dentitions. Concurrence of DE and DI within the same tooth is extremely rare. We report a case of DE and DI in a maxillary right lateral incisor tooth. Comprehensive clinical and radiographic examinations are essential to identify such defects; the early diagnosis can then result in the appropriate prophylactic treatment being performed, thus preventing undesirable pulpal complications.