The genetic pathology often determins a complex and extreme polimorphclinical aspects. Among the frequently found anomalies are those in thedentomaxillary field. The Genetic Department from "Louis Turcanu"Children's Emergency Hospital investigated and observed between 2000-2005,540 children with genetic diseases. 78% of them presented from minor tomajor dentomaxillofacial anomalies. Among the registated cases cromosomialsyndroms, monogenic diseases, different caused plurimalformative syndromswere found. For the dentomaxillar anomalies diagnosis the dentistry exam wasoften indispensable and highly important, revealing hardly detectablediseases. The early dental exam is necessary in all genetic syndroms, for acorrect topic and adequate therapeutical directions. The dentist completsthe multidisciplinary team which participates to diagnosis and observinggenetic diseases. Keywords: dental exam, genetic diseases, dentomaxillary anomalies