Heritable dentin defects form a group of diseases which exclusively affect dentin among the various dental tissues. While one type is associated with the generalized connective tissue disorder, osteogenesis imperfecta, other types occur as single traits. The clinical manifestations of the dentin defects vary from insignificant to severe enough to cause aesthetical and functional failure of the teeth. Scanning and transmission electron microscopic studies, reviewed in this paper, have markedly clarified the ultrastructure of the aberrant dentin matrix. Both similar and different changes seem to occur in the various forms of heritable dentin defects. Abnormalities in the appearance and organization pattern of collagen fibers in the defective dentin partly resemble those observed in skin in generalized connective tissue diseases. The similarity of ultrastructural findings in dentin defects, which are currently classified as distinct entities, and even in diseases affecting other tissues, could be related to the complicated interactions between the extracellular matrix macromolecules. Thus, many of the changes observed may be secondary in nature. Ultrastructural studies can help us to understand the pathogenesis of the different types of heritable dentin defects as well as aid in diagnostics and classification of these diseases.