Introduction and Objective. Tooth agenesis may be present even in 9.6% of individuals of both genders, with the predominance in permanent dentition. The aim of the study was to present a review of the literature on the etiology of dental agenesis, with particular emphasis on the genetic background and the associated syndromes of congenital abnormalities. Objective and Review Methods. Online databases were searched: Pubmed, GoogleScholar and Scopus. 32 articles from the last 18 years were qualified for the study and used in this review. Inclusion criteria were: children, congenital disorders, syndromic diseases, missing teeth, tooth agenesis. The study takes into account both syndromes common in the population and rare disorders. Brief description of the state of knowledge. Many genes are responsible for both isolated and syndromic tooth agenesis. The most studied and involved in the formation of this anomaly are: MSX1, PAX9, AXIN2, WNT10A, EDA, TGF, SHH. The presented study shows that various forms of agenesis are caused by genes that involve interacting molecular pathways. The course of this phenomenon is very diverse, especially in syndromes of defects accompanied by other craniofacial anomalies. Summary. The absence of one or several teeth seriously disrupts the proper development of a child, as tooth agenesis affects the craniofacial development and psychosomatic development of a patient at developmental age. Children diagnosed with missing tooth buds should receive comprehensive treatment, which includes dentists with such specialties as: maxillofacial surgeon, dental surgeon, orthodontist or prosthodontist.