Dens in dente, also known as dens invaginatus and dilated compound odontoma, is a malformation that can occur on primary, permanent, or supernumerary teeth that is characterized by a deep invagination of the surface of a crown or root covered with enamel. This abnormality in tooth morphology generally affect the maxillary lateral incisors but several cases of multiple dens invaginatus have been reported in the literature. A 15 year-old female patient is reported here presenting five dens invaginatus: four in the permanent mandibular incisors and one in the permanent, maxillary left central incisor, additionally the following dental findings were observed: a permanent mandibular left mulberry molar, molarization of some premolars, several microdontic conoid teeth, retention of five primary teeth, absence of several permanent teeth germs, a macrodontic molar with abnormal roots and several periapical radiolucencies associated to the dens invaginatus. There was no family history of similar dental findings to those observed in the patient. There are several genes that participate in the development of teeth, of those, the following five genes could be implicated as responsible or co-participators for some of the dental anomalies present in this patient: MSX1 (Muscle segment homeobox 1), DLX1 and DLX2 (Distal-less homeobox 1 and 2 genes), PAX9 (Paired box gene) and PITX2 (Pituitary homeobox transciption factor 2).