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A 12-year-old boy with characteristic findings of otodental syndrome is presented. Abnormalities of deciduous and permanent dentition included bulbous canines, globe-shaped posterior teeth and agenesis of maxillary premolars. High-frequency sensorineural hearing loss was demonstrated, and results of genetic studies indicated an autosomal dominant trait. This patient presented previously unreported findings, including generalized macrodontia, delayed mineralization of the mandibular premolars, and supplementary permanent maxillary canines.