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Twelve persons in four generations of a rural family of mixed racial ancestry exhibited ankylosed teeth, bilateral clinodactyly of the fifth fingers, and abnormalities of the jaws. This previously undescribed association of dental, digital, and skeletal anomalies appears to be transmitted in an autosomal dominant manner in this family. Clinical manifestations in the examined persons and an extended family history are described in detail. Previous studies of apparently hereditary dental ankylosis are similarly discussed.

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