Dyskeratosis congenita (DC) is an inherited bone marrow failure syndrome that is 
characterized by lacey reticular hyperpigmentation of the skin, dystrophic 
nails, mucous membrane leukoplakia and pancytopenia. Diagnosis may be delayed 
until clinical signs are apparent. Severe pancytopenia frequently causes early 
mortality of DC patients, who have an increased risk of developing oropharyngeal 
squamous cell carcinoma. Several case reports have described oral changes in DC, 
which include oral leukoplakia, increased dental caries, hypodontia, thin enamel 
structure, aggressive periodontitis, intraoral brown pigmentation, tooth loss, 
taurodontism and blunted roots. We determined the prevalence of these previously 
reported findings in a cohort of 17 patients with DC and 23 family members. The 
most common oral changes in DC patients were oral leukoplakia (65% of the entire 
DC population), decreased root/crown ratio (75% with sufficient tooth 
development) and mild taurodontism (57% with sufficient tooth development). From 
the clinical perspective, a diagnosis of DC or other inherited bone marrow 
failure syndrome should be considered in young persons with oral leukoplakia, 
particularly those with no history of smoking. Multiple permanent teeth with 
decreased root/crown ratios further suggest DC.