FA syndrome is a genetic disorder caused by germline biallelic mutations in one of 13 genes (including FANCD1/BRCA2 and FANCN/PALB2) of the so-called FA/BRCA pathway; this pathway controls the repair of double strand-breaks and the response to DNA crosslinking agents [3,4]. The gene discussed is BRCA2; the disease is Friedreich ataxia.