Dens evaginatus (DE) and dens invaginatus (DI) are developmental anomalies that affect the shape of the teeth. Although they have been found singly and in association with other developmental anomalies in the same patient, it is uncommon to observe these two anomalies in the same tooth. There are only a few published cases of concurrent DI and DE in the same tooth. The patient in the case reported here was a 27-year-old man who exhibited a novel combination of DE and DI in permanent maxillary incisors. Early diagnosis of this anomaly is necessary. The clinician then can take particular care when performing prophylactic odontoplasty for the DE, keeping in mind that the associated DI presents an increased risk of early pulpal involvement. This article focuses on the management aspects of DE and DI.