Fanconi anemia (FA) is a rare (1:160,000), principally autosomal recessive hereditary disease (except for FANCB and FANCR/RAD51) (Figure 1); it develops due to biallelic germline downregulation of any one of the 22 currently identified FANC complementation genes (FANCA–FANCW) [1,2,3,4,5]. The gene discussed is FANCA; the disease is Friedreich ataxia.