Abstract
Oculomandibulodyscephaly (OMD) is characterized by anomalies of skull, mandible, skin and eyes and also by a proportionate nanism. Dental abnormalities are often observed but poorly defined. Examination of a 2‐month‐old infant that suffered from OMD, having died from an unrelated cause revealed premature eruption of several deciduous teeth, crowding of unerupted deciduous teeth and agenesis of all permanent teeth excepting the first permanent molars.

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