As the pedodontists rather frequently encounter cases of the congenital abnormalities or developmental anomalies in their daily practice, it is essential that they should possess an adequate knowledge of those anomalies in order to give proper diagnosis and establish effective treatment planning. Recently, we have observed a case of pycnodysostosis in a ten-year-six-month old girl, whose dental characteristics provide some additional findings to those already pointed out by previous investigators in the field. The pycnodysostosis was first defined by MAROTEAUX-LAMY [ 1 ] as an independent entity which differs from Albers-Schonberg disease in points of the anomaly of cranial, peripheral bone and the mandible. Since MAROTEAUX-LAMY first reported on this in 1962, other case reports were followed by SHULER [ 2 ] in 1963, STANESCO As the first effort by a domestic researcher, the one by MURAKAMI et al. [ 5 ] was the presentation of systemic findings on the same patient as reported in this paper. Case History
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