Biallelic germline alterations in one of the 22 genes implicated in the so-called “FA pathway” (like BRCA1, FANCD2, SLX4/FANCP and FANCJ) are the cause of this rare genetic disease, characterized by cellular hypersensitivity to DNA cross-linking agents, while its main clinical features are retarded growth, abnormalities of the skeletal system, bone marrow failure, development of acute myeloid leukemia and solid tumors (including HNC) at a young age [15]. The gene discussed is BRIP1; the disease is Friedreich ataxia.