Dentinogenesis imperfecta is a hereditary disorder resulting in defective d
entin in both the primary and secondary dentitions. The complications of de
ntinogenesis imperfecta are difficult to manage and provide a challenge to
the dentist. This case report concerns treating an African American patient
with dentinogenesis imperfecta who appeared for treatment with endodontic
pathosis. It illustrates the need for appropriate and timely restorative tr
eatment to prevent pulpal pathosis. Also demonstrated is the difficulty of
endodontically treating dentinogenesis imperfecta teeth because of pulpal o
bliteration and abnormal dentin mineralization. Early and correct diagnosis
of dentinogenesis imperfecta is imperative to enable appropriate preventiv
e interventions and optimal dental treatment. Although pulpal pathosis is r
arely reported with dentinogenesis imperfecta, endodontic treatment is occa
sionally necessary and has a guarded prognosis if initiated after pulp cana
l obliteration has occurred.