Fanconi’s Anemia (FA) is a rare autosomal recessive disorder characterized by thrombocytopenia,diverse congenital malformations which include skeletal malformations, hyperpigmentation, urogenital, renal and cardiac anomalies. We report a case of Fanconi anemia who presented with thrombocytopenia, bilateral hypoplastic thumbs, café-au-lait spots, with severe bleeding from the gingiva managed with platelet transfusion and extraction of the tooth was done