Introduction and Objective: Dyskeratosis congenita (DC) also known as Zinsser-Engman- Cole Syndrome is a rare inherited disorder with a prevalence of less than one per million. Zinsser et al. described an inherited variant of ectodermal dysplasia that affected skin, nails and mucous membranes in early 1900s.The syndrome eventually came to be known as DC and is classified as one of the inherited bone marrow failure syndromes (IBMFS). DC is the association of three clinical features: dystrophic nails, oral leukoplakia (white spots on the tongue and oral mucosa) and abnormal skin pigmentation. Case report and Conclusion: This case report describes a dental management of a case of DC. Fluctuating vital and blood parameters and deteriorating overall health status were major challenges delivering dental treatment. Dental extractions of this patient were done while maintaining blood parameters by blood and platelet transfusion.