Sotos syndrome, also called cerebral gigantism due to cranial overgrowth, is characterized by fast growth in the first stage of life. It is usually transmitted by autosomal dominant inheritance; nevertheless, recessive forms have been reported in the literature. Cole and Hughes established 4 principal criteria: overgrowth with advanced bone age, macrocephaly, characteristic facial appearance, and learning difficulties. Recently, mutations and deletions in the gene NSD1 have been identified as the main cause of the syndrome. The prevalence has been estimated to be 1 per 10–50 000. The oral findings are varied: high-arched palate, premature eruption of teeth, mandibular prognathism, agenesis, supernumerary teeth, and limited mouth opening.

This report describes the planning of oral rehabilitation of a patient with Sotos syndrome presenting with multiple dental agenesis. To our knowledge, there have been few cases of Sotos syndrome with agenesis described in the literature.2,4  Replacement...

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