Key Clinical MessageSuccessful management of a rare case involving both dens evaginatus and dens invaginatus in the same tooth, monitored over a 24‐month follow‐up.AbstractDens invaginatus (DI) is a congenital dental anomaly characterized by the presence of a tooth that resembles a “tooth within a tooth.” Conversely, dens evaginatus (DE) is a developmental anomaly distinguished by an additional tubercle or cusp on the tooth's crown. Both DI and DE can result in pulpal and periapical diseases in the affected tooth. This article presents a case of a healthy 14‐year‐old male with recurrent swelling under the chin and a wound with pus drainage on the right side of the submental area, associated with his left mandibular lateral incisor affected by both DI and DE. Clinical and radiographic examinations revealed that the tooth was necrotic and had a chronic apical abscess. Cone beam computed tomography (CBCT) confirmed Oehlers' type III DI and a talon cusp on the lingual surface of the same tooth. The patient underwent orthograde endodontic treatment. Passive ultrasonic activation of a 5.25% NaOCl solution and a mixture of Ca(OH)2 and a 2% chlorhexidine solution were utilized to effectively clean and eliminate the persistent pus discharge. After the resolution of the patient's symptoms, the apical third of the root canal and the invaginated space were filled with a plug of mineral trioxide aggregate (MTA), while the remaining root canal was filled using a sealer‐based obturation technique. A 24‐month follow‐up visit revealed complete bone regeneration in the previously affected periradicular tissues.