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The case of a young patient with a lingual dermoid cyst is presented. This case is unusual for the occurrence of the mass within the tongue, for its lateral localization, and for the association with other minor anomalies such as hypomelanotic areas, synophrys, hypertelorism and low-set and poorly structured ears. Bilateral broad, short fifth metacarpals with low-set fifth fingers of the hands were also present. A full clinical history, instrumental data, subsequent operative procedures and hystologic examination of the cyst, are described.
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