Abstract

This report documents a unique case of rough hypoplastic amelogenesis imperfecta with apparent anterior oligodontia and multiple anomalies of the associated mesenchymally derived tissues. Multiple unerupted teeth showed hypercementosis, distorted roots with aberrant dentin formation, and marked follicular hyperplasia. The hyperplastic follicles had a complex histopathologic appearance that recapitulated some features of the WHO-type odontogenic fibroma. The features of these teeth, the nature of the associated follicular lesions, and their relationship to the unerupted teeth are discussed.

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