We showed in this communication a peculiar case of mucopolysaccharidosis which subtype is unknown in a 16-year-old male. The symptoms observed inn this patient included short stature, kyphosis, joint stiffness, mental retardation, as well as increased excretion of urinary dermatan sulfate. Radiologic changes of dysostosis multiplex involved the spine, ribs, pelvis, and hands. Acroosteolysis of all distal phalanges, previously undescribed, was seen. Radiograms of jaw bones showed flattening of

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... lateral mandibular heads, multiple dentigerous cyst-like radiolucent lesions in both upper and lower molar regions, a number of carious lesions, enlarged pulp chamber and delaceration of the roots. Cephalometric analysis revealed marked underdevelopment of the mandible.