Individuals with mutations in any of the 23 FANC protein-coding genes necessary for ICL repair develop Fanconi anemia, although mutations in FANCA are the most common (2–8).The spectrum of phenotypes in Fanconi anemia patients is broad and includes developmental abnormalities, bone marrow failure, infertility, and profound cancer predisposition, primarily to acute myeloid leukemia and mucosal squamous cell carcinomas (SCCs) including head and neck, esophageal and anogenital (2, 5, 6, 8, 9). The gene discussed is FANCA; the disease is Fanconi anemia.