Regional odontodysplasia is an uncommon dental anomaly that is not hereditary and has a still unknown etiology. The affected tissues are enamel and dentin, the primary teeth are affected, and sometimes in the permanent dentition of the same individual. This lesion may be confused with other lesions such as odontoma or teeth with another type of anomaly. The objective is to report a case of regional odontodysplasia in the deciduous and permanent dentition of a child, as well as to present the different diagnostic tools. The affected teeth showed clinical examination, such as hypoplastic teeth with a yellow-brown coloration. A panoramic radiograph and periapical radiographs, as well as an excisional biopsy, were essential to obtain a correct diagnosis, so as to be able to offer an adequate conduct for the preservation and predictability of the different types of dental rehabilitation...