Despite the small number of patients, which, is a limitation of this study, we conclude that FA in Egyptian children is associated with an up regulation of p53 gene mutation which could explain the tendency of those patients to develop malignancies, on the other hand the AAA, only one case (10%) showed the expression of the mutant p53, this case has a severe hypoplastic marrow which postulate the possible oncogenic cause in AAA. Here, TP53 is linked to Friedreich ataxia.