Malformations of the tongue, are structural defects, present at birth and happening during embryogenesis. Developmental anomalies or defects may be major or minor, single or multiple, depending on their size, site and effect. The aim of this study was to present and to describe congenital syndromes which are associated with tongue malformations and to classify these malformations in groups, according to the tongue's clinical manifestations. The most common malformations of the tongue combined with syndromes associated with them, are fully discussed in this review article. Malformations of the tongue which are discussed in this review article, have been classified in the following categories: 1. Aglossia 2. Microglossia 3. Tongue hemiatrophy 4. Tongue hemihypertrophy 5. Macroglossia 6. Long tongue 7. Ankyloglossia 8. Cleft or bifid tongue 9. Syndromes that affect tongue and cannot be classified in a special condition Each category is discussed separately in this article.