Özet:

Sotos syndrome is a genetic condition characterized by excessive growth, macrocephaly, specific facial appearance, and varying degrees of learning disability without an endocrine disorder in childhood. Most of the cases were sporadic and the families who matched the autosomal dominant inheritance pattern were reported. The gene responsible for the syndrome encodes the nuclear receptor binding SET domain1 (NSD1) protocol. This rare genetic syndrome was first described by Sotos et al. In 1964 in five cases with excessive height, acromegalic appearance and moderate mental retardation. The high forehead hairline, macrocephaly, frontal bossing, long and thin facial appearance, hair sparse in the fronto- temporal region, downward slanting palpebral fissures and prominent mandible are the characteristic facial appearance, and the advanced bone age and mental retardation in different levels are the other diagnostic criteria.The aim of this case report is to emphasize the dental management of 6 year old sotos syndrome with common dental caries and specific oral findings.

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