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Management of bleeding disorders poses a challenge to dentists. These conditions can be inherited, acquired, or congenital are associated with an increased risk of hemorrhage, poor wound healing, and infection. Fanconi anemia (FA) is a rare autosomal recessive disease which belongs to a group of chromosome break up or DNA repair disorders. Hematologic abnormalities represent the most prevalent pathologic manifestation of FA. Herein, we report a case of FA in which various classical signs of the disease were present. We have given emphasis on oral manifestations and the role of a pedodontist in making an early diagnosis and providing comprehensive care for the maintenance of oral health of the affected individuals.