Although MSI2 was shown to be a dependency of aggressive myeloid leukemias, as well as solid cancers such as pancreatic cancer (Fox et al., 2016; Ito et al., 2010; Kwon et al., 2015; Kharas et al., 2010), and its transgenic overexpression can lead to increased tumor burden (Kharas et al., 2010), whether MSI2 translocations or mutations harbored in patients can serve as oncogenes and driver mutations remains unknown. Here, MSI2 is linked to familial pancreatic carcinoma.