<![CDATA[Agenesis of teeth is a developmental anomaly of the stomatognathic system characterized by the congenital absence of one or more teeth due to the failure of tooth germ formation during odontogenesis. This condition may affect both primary and permanent dentition and is classified according to the number of missing teeth into hypodontia, oligodontia, and anodontia. The method used in this report was a case report accompanied by a literature review based on relevant data sources. The case involved a 16-year-old female patient who presented with complaints of multiple spaces between her teeth and dental caries. Further examination, including panoramic radiography, revealed the congenital absence of nine permanent teeth: 17, 15, 24, 25, 27, 37, 35, 44, and 47. Clinical examination also showed the persistence of several primary teeth (55, 63, 65, and 75) with dentinal carious lesions but without mobility. This condition may result from mutations in genes involved in tooth development, such as MSX1, PAX9, and AXIN2. Mutations in these genes can disrupt epithelial-mesenchymal interactions during odontogenesis, leading to failure of tooth germ formation and resulting in multiple tooth agenesis. Oligodontia requires individualized management because its clinical manifestations vary considerably depending on the number of missing teeth, the condition of the alveolar bone, occlusal relationships, and the patient's age. Therefore, a multidisciplinary approach involving orthodontists, oral and maxillofacial surgeons, prosthodontists, and, when necessary, other dental specialists is essential to achieve optimal functional and esthetic outcomes.]]>
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