Fanconi Anemia (FA) is an extremely rare autosomal recessive disorder characterized by chromosomal break up that induces congenital abnormalities. FA results from a mutation in one of the 15 genes involved in the DNA repair pathway that is essential for the proper development of white blood cells, red blood cells, and platelets (Dental Perspective of Rare Disease of Fanconi Anemia (2). Some signs made up of a short stature, hyperpigmentation and bone marrow failure should suggest the diagnosis. In this paper we report two case reports of FA of in different ages who were followed in our Paediatric Dentistry Department in la Rabta Hospital and in which various classical signs were present. Comparing the different symptoms, we noticed that developmental and physical abnormalities are in common such as hyperpigmentation, short stature, skeletal abnormalities and some oral manifestations such as microdontia, periodontitis, and dental caries. Due to an increased risk of malignancies in this population, we have given emphasis on oral manifestations and the role of pediatric dentist in making early diagnosis and ensuring the maintenance of oral health for these patients.