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Examining the variation of the number of teeth agenesis of permanent teeth is relatively common but real oligodontia is rarely encountered during our daily work. At a number of patients recently attending the Pedodontics and Orthodontics Department of the Dental Faculty of Semmelweis University oligodontia, the absence of at least six or more permanent teeth was found. Examining the aetiology of oligodontia it can be determined that both endogen and exogenous environmental factors may contribute to the cause of the anomaly. In its pathology genetics is considered as the dominant factor what is also proved by familiar inheritance aggregation however it is hard to determine the aetiology in most cases. Severe oligodontia most often is part of certain syndromes. The anomaly causes not only aesthetical and functional problems but also may lead to several complications so early recognition and referral is considered essential. Therapy often involves multidisciplinary approach with individual assessment and planning. 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