A 15-year-old female with a clival conventional chordoma tested positive for a heterozygous pathogenic mutation in the FANCA gene (c.2852G > A/p.Arg951Gln); her family history was significant for a maternal aunt with early onset breast cancer with negative BRCA1/2, and maternal grandmother with pancreatic cancer. This evidence concerns the gene BRCA1 and familial pancreatic carcinoma.