Küry et al. point out that, to their knowledge, “BAP1 is one of the rare genes in which different germline variants cause either a neurodevelopmental disorder or a tumor predisposition syndrome.” Future studies are warranted to investigate precise mechanisms through which BAP1 regulates ENS development and differentiation, and to identify additional BAP1 roles in the nervous system. Here, BAP1 is linked to BAP1-related tumor predisposition syndrome.