This case report illustrates type II dens invaginatus (DI) and analyzes the possible aspects of this defect. Dens invaginatus is a developmental anomaly of teeth that commonly affects permanent maxillary lateral incisors. This case report focuses on the non-surgical and surgical management of right maxillary lateral incisor associated with type II DI. Pulpal involvement of malformed tooth, bizarre in root canal anatomy, periapical pathosis and severe periodontal destruction were noticed. Endodontic therapy with combined obturation techniques, surgical therapy with apical-curettage, retrofilling with glass ionomer cement and bone grafting were performed. A year follow-up evaluation showed satisfactory periapical healing.