Recurrent mutations in the highly conserved HEAT 5–9 repeats of splicing factor 3B subunit 1 (SF3B1) have been reported in myelodysplastic syndrome, chronic lymphocytic leukemia (CLL), breast cancer (BRCA), uveal melanoma (UM), and pancreatic cancer [2–7]. The gene discussed is SF3B1; the disease is familial pancreatic carcinoma.