The inconspicuous cytogenetic and comparatively mild FA‐like clinical phenotype presented here is most likely due to incomplete impairment of BRCA1 function by the p.Arg1699Gln risk allele, which was described to intermediately increase BC/OC risks by the age of 70 years to 20% and 6%, respectively (Moghadasi et al., 2018; Spurdle et al., 2012). Here, BRCA1 is linked to Friedreich ataxia.