Even though the family was otherwise asymptomatic, because the Fanconi anemia (FA) pathway is a well-known leukemia predisposing disorder and FA-associated gene dysfunction has been linked to genomic instabilities, defects in Holliday junction resolution [13] and aneuploidy [14], we postulated that inherited rare disadvantaging DNA variants in FA cancer predisposing genes/pathway, in combination with PRDM9, could contribute to the chromosome instabilities underlying this case of familial hyperdiploid childhood ALL. The gene discussed is PRDM9; the disease is Friedreich ataxia.