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18p- syndrome is caused by the deletion of all or a portion of genetic material on the short (p) arm of chromosome 18. It was first described in 1963 and since then there have been a variety of clinical characteristics associated with this syndrome. The patient described presented with learning difficulties, epilepsy, a characteristic round face and anti-mongoloid slant to the eyes. Orally he had a single maxillary central incisor and a high caries rate which necessitated comprehensive dental treatment under general anesthesia.