Regional odontodysplasia is an uncommon developmental anomaly affecting a localized area of dentition, with distinctive clinical, radiographic, and histological findings. This article reviews a case of a 14-year-old female who reported with unerupted maxillary anterior teeth. This case was rare in that it involved maxillary dentition with unerupted maxillary anterior teeth on the right side, but the left central incisor was missing. The molars on the right side were showing some amount of abnormality. Radiographically, the affected teeth had a ‘ghostly’ appearance, showing a marked reduction in radiodensity. Both enamel and dentin appeared to be very thin, the pulp chamber was exceedingly large, and the roots were short with wide open apices. All the characteristics were consistent with the diagnosis of regional odontodysplasia. The care and treatment of this patient required a multidisciplinary approach. The unerupted maxillary anterior teeth were surgically removed, following which temporary prosthetic restoration was provided to improve esthetics and to restore the function.

Adenomatoid Odontogenic Tumor (AOT) is a well-established benign epithelial lesion of odontogenic origin. Rightfully called “the master of disguise,” this lesion has been known for its varied clinical and histoarchitectural patterns. Not only does AOT predominantly present radiologically as a unilocular cystic lesion enclosing the unerupted tooth (which is commonly mistaken as a dentigerous cyst) but the lesion also presents rarely with a cystic component histopathologically. We present one such unusual case of cystic AOT associated with an impacted canine, mimicking a dentigerous cyst. The present case aims to highlight the difference between cystic AOT and dentigerous cyst radiographically. The exact histogenesis of AOT and its variants still remains obscure. An attempt has been made to hypothesize the new school of thought regarding the origin of AOT.