In 16/60 (26%) of the SPS patients, we found variants in genes encoding for proteins of the Fanconi Anemia (FA) pathway (BLM, BRCA1, FAN1, FANCA, FANCD2, FANCL, PALB2, RAD50, RAD51C, RAD51D), that are involved in the DNA Interstrand-Cross Link repair (DNA-ICLR). Here, FANCA is linked to Friedreich ataxia.